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T year__Core conditions__this week confident__T__Haematology Flashcards

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1
Q

What is microcytic anaemia&nbsp

A

<ul><li>Defined as small hypochromic RBC's with low mean corpuscular volume ~ typically below 83 microns&nbsp
</li></ul>

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2
Q

List some causes of haemolytic anaemia&nbsp

A

Iron deficiency anaemia&nbsp

</li><li>Insufficient absorption&nbsp

</li><li>Increased iron loss&nbsp

</li><li>Increased iron requirements&nbsp

</li></ul><div><br></br></div>Sideroblastic anaemia&nbsp

<br></br><ul><li>Lead poisoning&nbsp

</li><li>Congenital&nbsp

</li><li>Chronic alcohol absue&nbsp

</li><li>Anti TB meds</li><li>Myelodysplasic syndromes</li><li>Alpha and beta thalassaemia&nbsp

</li><li>Anaemia of chronic disease ~ normocytic</li></ul>

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3
Q

List some general signs and symptoms of anaemia&nbsp

A

<ul><li>Tiredness&nbsp

## Footnote

</li><li>Headches/dizziness</li><li>Conjunctiva pallor&nbsp

</li><li>Tachycardia</li><li>Increased RR</li><li>Dyspnoea&nbsp

</li><li>Cold intolerance&nbsp

</li></ul>

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4
Q

List some specific signs and symptoms of iron deficiency anaemia&nbsp

A

<ul><li>Koilonychia&nbsp

## Footnote

</li><li>Pica ~ unusual dietary cravings&nbsp

</li><li>Atrophic glossitis&nbsp

</li><li>Angular stomatitis&nbsp

</li></ul>

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5
Q

List some signs of lead poisoning (causes sideroblastic anaemia)&nbsp

A

<ul><li>Motor Peripheral Neuropathy (e.g: Reduced grip strength, weakness in muscles/ absent reflexes)</li><li>Bowel Disturbance</li><li>Confusion/personality change</li><li>Metallic taste in the mouth</li><li>Haemolysis (clinically presenting with dark urine and pallor)</li><li>Blue line on gum and dense metaphysial lines on radiograph</li></ul>

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6
Q

Outline the investigations for iron deficiency anaemia&nbsp

A

<ul><li>FBC&nbsp

## Footnote

</li><li>Peripheral blood smear ~ demonstrates <b>hypochromic microcytic</b> anaemia&nbsp

</li><li>Reticulocyte count ~ low&nbsp

</li><li>MCV ~ less than 83 microns&nbsp

</li></ul>

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7
Q

What are the investigations for new iron deficiency anaemia&nbsp

A

<ul><li>Colonscopy&nbsp

## Footnote

</li><li>Need to be investigated for cancer</li></ul>

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8
Q

Outline the invesitgations for sideroblastic anaemia&nbsp

A

<ul><li>FBC&nbsp

## Footnote

</li><li>Iron studies ~ high ferritin, iron and transferrin&nbsp

</li><li>Blood films&nbsp

</li><li>Bone marrow examination&nbsp

</li></ul>

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9
Q

Outline the management of iron deficiency anaemia&nbsp

A

<ul><li>Treat underlying cause&nbsp

## Footnote

</li><li>Use erythropoietin if ferritin and transferrin saturation is normal&nbsp

</li></ul>

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10
Q

Outline the management of sideroblastic anaemia&nbsp

A

<ul><li>Treat underlying cause&nbsp

## Footnote

</li></ul>

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11
Q

What is macrocytic anaemia&nbsp

A

<ul><li>When the RBC are bigger than normla but there is still low Hb&nbsp

## Footnote

</li></ul>

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12
Q

What is megaloblastic macrocytic anaemia&nbsp

A

<ul><li>There is slow or impaired DNA synthesis which lead to delayed maturation of RBCs&nbsp

## Footnote

</li><li>There is also <b>hypersegmented neutrophils </b>on blood film&nbsp

</li></ul>

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13
Q

List some causes of macrocytic&nbsp

A

anaemia Megaloblastic:<br></br><ul><li><b>B12 deficiency </b>~ pernicious anaemia, dietary insufficiency and malabsorption&nbsp

</b></li></ul>Normoblastic<br></br><ul><li>Alcohol - accompanied by raised yGGT</li><li>Reticulocytosis&nbsp

</li><li>Hypothyroidism&nbsp

</li><li>Liver disease</li><li>Drugs&nbsp

</li><li>Myelodysplasia&nbsp

</li></ul>

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14
Q

List some causes of B12 deficiency&nbsp

A

<ul><li>Vegan diet&nbsp

## Footnote

</li><li>Illeal resection&nbsp

</li><li>Crohns disease</li></ul>

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15
Q

List some causes of pernicious anaemia&nbsp

A

<ul><li><blockquote><div>40 years old</div></blockquote></li><li>Background of auto-immune thyroid disease, vitiligo, T1DM and Addison's disease</li><li>Female</li></ul>

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16
Q

List some causes of a folate deficiency&nbsp

A

<ul><li>Pregnancy (Increased demand)</li><li>Poor diet + Alcohol</li><li>Coeliac disease (Malabsorption)</li><li>Drugs</li></ul>

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17
Q

List some signs and symptoms of macrocytic anaemia&nbsp

A

<ul><li>Pale skin &amp

## Footnote

SOB</li><li>Tiredness</li><li>Headaches / Dizziness</li><li>Beefy tongue</li><li>Worsening of other conditions such as angina, heart failure or peripheral vascular disease</li></ul>

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18
Q

List some signs and symptoms of B12 deficiency anaemia&nbsp

A

<ul><li>Peripheral Neuropathy: Pins &amp

## Footnote

vibration sense&nbsp

or&nbsp

proprioception</li><h2></h2></ul>

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19
Q

Outline the investigations for macrocytic anaemia <ul><li>FBC&nbsp

A

</li><li>Haematinics ~ serum B12 and folate, MCV high</li><li>Blood film&nbsp

</li><li>LFT’s&nbsp

</li><li>Antibodies to intrinsic factor&nbsp

</li><li>Markers of haemolysis ~ bilirubin etc&nbsp

</li></ul>

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20
Q

Outline the management of megaloblastic anaemia&nbsp

A

<ul><li>IM hydroxocobalamin (VitB12)</li><li>Folic acid&nbsp

## Footnote

</li><li>You need to address the B12 deficiency first before folate replacement to avoid exacebating neuro symptoms and causing subacute degeneration of the spinal cord&nbsp

</li></ul>

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21
Q

Outline the management of normoblastic anaemia&nbsp

A

<ul><li>Treat the underlying cause like addressing alcohol consumption or support during pregnancy&nbsp
</li></ul>

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22
Q

What are some complications of megaloblastic anaemia&nbsp

A

<ul><li>Pernicious anaemia ~ increased risk of gastric cancer&nbsp

## Footnote

</li></ul>

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23
Q

What is sickle cell anaemia&nbsp

A

<ul><li>Genetic condition where normal haemoglobin has a tendency to form abnormal Hb molecules upon deoxygenation leading to distorted RBC's&nbsp

## Footnote

</b></li><li><b>HbSC - milder</b></li><li><b>HbAS - trait</b></li></ul>

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24
Q

What causes sickle cell disease&nbsp

A

<ul><li>Autosomal recessive inheritence ~ HbSS instead of HbAA</li><li>HbSS ~ severe form&nbsp

## Footnote

</li></ul>

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25
Outline the pathophysiology of sickle cell disease 
  • At the 6th position of the beta chain, glutamic acid is replaced by valine - decreases water solubility of deoxyHb
  • In its deoxygenated state, the HbS undergoes polymerisation to form cystals that cause polymers to form - RBC become sickle shaped 
26
What are the signs and symptoms of sickle cell disease 
  • Symptoms dont show till after 4-6months of birth as there is transition from foetal Hb to adult Hb  ## Footnote
  • Splenomegaly - due to extravascular haemolysis in the spleen 
  • Progressive anaemia 
  • Acute chest crises ~ tachypnoea, wheeze, couhg, hypoxia and pulmonary infiltrates on CXR
27
Outline the investigation for sickle cell disease
  • Newborn screening programme ~ heel prick on day 5 after birth 
  • Haemoglobin electrophoresis ~ definitive diagnosis  ## Footnote
  • Blood film ~ sickle cells, target cells, reticulocytosis with polychromsia, features of hyposplenism(Howell Jolly bodies, nucleated RBC)
    • 28
    Outline the management of chronic sickel cell disease 
    Chronic SCD
    • Hydroxycarbamide/hydroxyurea ~ increases foetal Hb conc  ## Footnote
    • Bone marrow transplant 
    • Blood transfusion, folic acid and iron chelation 
    29
    Outline the emergency presentation of acute sickle cell crisis
    • Painful crisis
    • Vaso-oclusive crisis- happens in the cold
    • Splenic sequestration crisis ~ RBC block splenic blood flow to cause splenomegaly and abdo pain, inc reticulocyte count
    • Acute chest syndrome ~ vaso-occlusion in the pulmonary micorvasculature to cause infarcation of lung parenchyma 
  • Aplastic crisis ~ temporary loss of RBC production which is triggered by parvovirus B19 infection, reduced reticulocyte count and sudden fall in Hb
  • Haemolytic crisis ~ fall in Hb is related to rasied reticulocytes and prehepatic hyperbilirubinaemia 
    • 30
    Outline the emergency management of acute sickle cell crisis
    • IV analgesia 
  • High flow oxygen  ## Footnote
  • Antibiotics 
  • Blood transfusion 
  • Exchange transfusion ~ remove blood and replace with donor blood 
    • 31
    List some complications of sickel cell disease 
    • Sickle cell crisis  ## Footnote
    • Anaemia 
    • Avascular necrosis in large joints 
    • Stroke 
    • Pulmonary HT
    • CKD 
    • Hyposplenism ~ sickle cells get stuck in the spleen and undergo phagocytosis to cause splenic congestion and splenomegaly - compromised splenic function therefore prone to infections
    32
    What is thalassaemia 
    • Group of recessive inherited disorders characterised by abnormal Hb production 
    33
    What are the 2 types of thalassaemia 
    • Alpha ~ defect in the 4 genes for alpha globin chain 
    34
    Outline the pathophysiology of alpha thalassaemia 
    • Caused by nonfunctioning copies of the 4 alpha globin genes on chromosome 16
    • If they have 2 defective genes ~ pt has alpha thalassaemia trait  ## Footnote
    • If they have 3 defective copies ~ pt has symptomatic haemoglobin H disease (microcytic anaemia, haemolysis, splenomegaly)
    • If they have all 4 defective copies - they have hydrops fetalis which is incompatible with life 
    35
    What are the signs and symptoms of alpha thalassaemia 
    • Juandice  ## Footnote
    • Facial bone deformities 
    • Splenomegaly 
    36
    What are the investigations for alpha thalassaemia 
    • FBC ~ shows microcytic anaemia with disproportionately low MCV
    • Hb electrophoresis 
    37
    Outline the pathophysiology of beta thalassaemia 
    • If the pt has one abnormal of the 2 beta globin copies ~ beta thalassaemia minor 
    38
    List the signs and symptoms of beta thalassaemia major 
    • Severe symptomatic microcytic anaemia at 3-9 months  ## Footnote
    • Maxillary overgrowth 
    • Hepatosplenomegaly
    • Extramedullary hematopoiesis 
    • Failure to thrive 
    39
    Outline the investigations for beta thalassaemia major 
    • FBC ~ microcytic anaemia - disproportiante decrease in Hb and MCV - MCV much more decreased than iron
    • Hb electrophoresis  ## Footnote
    • Blood film ~ hypochromic microcytic cells, target cells, nucleated RBCs 
    • Pregnant women are offered screening tests 
    40
    Outline the management of thalassaemias 
    • Blood transfusions ~ beta major should have regular transfusion (2-4wks) of packed RBCs for the rest of their lives  ## Footnote
    • Stem cell transplant/ bone marrow transplant 
    • Reduce the risk of iron overload in beta major pts ~ give iron chelating agents like desferrioxamine
    41
    List some complications of beta thalassaemia 
    • Iron overload toxicity ~ due to recurrent blood transfusions  ## Footnote
    • Acute sepsis 
    • Liver cirrhosis
    • Endocrine dysfunction 
    42
    What happens to the bone marrow due to thalassaemia 
    • RBCs are more fragile and break down more easily
    • The spleen filters the defected RBCs and destroys them → splenomegaly
    • Bone marrow expands to compensate for the anaemia → increased susceptibility to fractures
    43
    What is haemolytic anaemia 
    • The premature destruction of RBCs resulting in low Hb conc
    44
    What are the 2 types of autommine HA
    • Warm 
  • Cold
    • 45
    What is warm AIHA
    • Haemolysis occurs at warm temperatures via antibody reaction 
  • IgG mediated  ## Footnote
    • 46
    What causes warm AIHA
    • Idiopathic 
  • SLE
  • Lymphoproliferative neoplasms  ## Footnote
    • 47
    What is cold AIHA
    • At lower temps, the AB's attach to the RBC
    s to clump them together ~ agglutination
  • The complement system is activated and the RBCs are destroyed  ## Footnote
    • 48
    What causes cold AIHA
    • Neoplasms 
  • Post infections - mycoplasma pneumoniae
  • Idiopathic 
    • 49
    List the key features of autoimmune haemolytic anaemia 
    • Anaemia  ## Footnote
    • Low haptoglobin 
    • High LDH and uncongugated bilirubin 
    • Blood film ~ spherocytes and reticulocytes 
    • Positive Coombs tests ~ direct antiglobulin test 
    50
    Outline the management of autommine haemolytic anaemia 
    • Steroids ~ prednisalone  ## Footnote
    • Blood transfusion 
    51
    What is ALL
    • A type of haemotological malignancy characterised by uncontrolled proliferation of immature lymphoid precursor cells within the bone marrow 
  • It is the most common cancer in childhood 
    • 52
    What causes ALL
    • Caused by the abnormal proliferation of lymphoid progenitor cells like B cells 
  • It is associated with Down syndrome 
    • 53
    List the subtypes of ALL
    • Precursor B cell ALL 
  • T cell ALL
  • B cell ALL
    • 54
    List some signs and symptoms of ALL
    • Fatigue 
  • Abnormal bleeding/bruising  ## Footnote
  • Bone pain 
  • Painless lymphadenopathy 
  • Hepatosplenomegaly 
  • Cranial nerve palsies 
  • Testicular enlargement
    • 55
    Outline the investigations for ALL
    • Bone marrow biopsy ~ diagnostic 
  • FBC - leukocytosis (WBC)
  • Blood film + bone marrow analysis ~ presence of 20%+ lymphoblasts/blast cells is diagnostic  ## Footnote
  • Lymph node biopsy 
    • 56
    What symptoms would warrant an urgent FBC to assess for leukaemia 
    • Pallor  ## Footnote
    • Unexplained fever 
    • Unexplained persistent or recurrent infection 
    • Generalised lymphadenopathy 
    • Unexplained bruising 
    • Unexplained bleeding
    • Unexplained petechiae 
    • Unexplained hepatomegaly 
    57
    Outline the chemotherapeutic regimen for ALL
    • Induction therapy ~ vincristine, prednisalone, L-asparaginase+daunorucibin 
  • Consolidation therapy ~ high/medium dose drugs in blocks over several weeks  ## Footnote
  • CNS prophylaxis ~ intrathecal methtrexate 
    • 58
    What is the supportive care for ALL
    • Blood/platelet transfusion 
  • IV fluids  ## Footnote
  • Insert subcut port system or Hickman line for IV access 
    • 59
    List some complications of ALL
    • Infections 
  • Bleeding  ## Footnote
  • Chemotherapy related toxicities 
    • 60
    What is AML
    • A haemotological malignancy characterised by uncontrolled proliferation of myeloid precursors in the bone marrow leading to bone marrow failure and the accumulation of immature WBC's in the peripheral blood 
  • Its the common acute leukaemia in adults
    • 61
    What causes AML
    • Deletion in chromosome 5 or 7 
  • Can result of a transformation from a myeloproliferative disorder
    • 62
    List some signs and symptoms of bone marrow failure 
    • Anaemia  ## Footnote
    • Neutropenia 
    • Splenomegaly 
    • Bone pain 
    63
    Outline the investigations for AML 
    • Bone marrow biopsy ~ diagnostic and will show a high proportion of blast cells, lots of immature myeloid cells  ## Footnote
    • Neutropenia and thrombocytopenia and anaemia
    • FBC 
    • Cytochemistry 
    • Cytogenetics
    • Immunophenotyping if its difficuly to distinguish between AML and ALL
    64
    Outline the medical management of AML 
    • Chemotherapy of daunorubicin and cytarabine but chemo starts with induction period and then consolidation therapy  ## Footnote
    65
    Outline the supportive care for AML 
    • Blood/platelet transfusion  ## Footnote
    • Allopurinol ~ prevents TLS
    • Insert subcut port line or Hickman line for IV access
    • Prophylactic antimicrobials 
    66
    What is CML
    • Its a myeloproliferative neoplasm characterised by the presence of the Philadelphia chromosome which leads to the formation of the BCR-ABL1 gene 
  • This altered gene results in an abnormal tyrosine kinase enzyme which causes the uncontrolled proliferation of myeloid cells in the bone marrow  ## Footnote
    • 67
    List some signs and symptoms of CML 
      ## Footnote
  • Tiredness 
  • Bleeding 
  • Gout 
  • May report a sense of fullness
  • Systemic symptoms ~ fever, sweating and weight loss
  • Hyperleukocytosis symptoms ~ visual disturbances, confusion, palpitation and deafness
    • 68
    What are the 3 phases of CML 
    • Chronic phase ~ lasts around 5 years, asymptomatic and is diagnosed incidentally due to raised WCC
    • Accelerated phase ~ abnormal blast cells make up the vast majority of cell in the bone marrow and blood, this is where they become symptomatic  ## Footnote
    69
    Outline the investigations for CML 
    • FBC ~ shows leukocytosis, neutrophilia, eosinophilia thrombocytosis/penia and anaemia  ## Footnote  
    • Peripheral blood smear ~ mature myeloid cells, and multiple basophils and oesinophils 
    • Blood film ~ increased granulocytes at different stages of maturation and thrombocytosis 
    • Bone marrow analysis 
    • Genetic testing for the BCR-ABL1 gene or Philadelphia chromosome (9,22)
    70
    Outline the management of CML 
    • Imatinib ~ first line drug and its a tyrosine kinase inhibitor  ## Footnote
    • Allogenic bone marrow transplant 
    71
    What is CLL
    • A haematological malignancy characterised by accumulation of mature monoclonal B lymphocytes in the blood, bone marrow and lymphoid tissues 
  • These abnormal B cells are often slow growing and crowd out healthy cells  ## Footnote
  • It may cause warm autoimmune haemolytic anaemia 
    • 72
    List some signs and symptoms of CLL
    • Typically asymptomatic 
  • Non tender lymphadenopathy  ## Footnote
  • Bleeding
  • Infection 
  • B symptoms ~ weight loss, night sweats and fever
    • 73
    Outline the investigations for CLL
    • Blood film ~ smudge cells, smear cells 
      ## Footnote
  • FBC ~ lymphocytosis - high lymphocyte count 
  • Bone marrow biopsy 
    • 74
    What is Richters transformation 
    • The transformation of CLL into high grade B cell lymphoma 
    75
    Outline the management of CLL
    • Asymptomatic ~ close observation 
  • Chemotherapy and steroids ~ rituximab + fuldarabine + cyclophophamide  ## Footnote
  • Support care ~ transfusion, IV human immunoglobulin if they have recurrent infection 
    • 76
    What are the complications of CLL
    • Anaemia
    • Hypogammaglobulinaemia ~ causes recurrent infections 
  • Warm autoimmune haemolytic anaemia 
    • 77
    List some complications of chemotherapy 
    • Failure to treat cancer
    • Stunted growth and development in children  ## Footnote
    • Neurotoxicity 
    • Infertility 
    • Secondary malignancy 
    • Cardiotoxicity 
    • Tumour lysis syndrome
    78
    What is tumour lysis syndrome 
    • When tumour cells are destroyed, all of thier contents are released into the bloodstream
    79
    What are the characteristics of tumour lysis syndrome 
    • High uric acid ~ can form crystals anywhere
    • High potassium ~ cardiac arrythmias
    • High phosphate
    • Low calcium 
    80
    How do you manage tumour lysis syndrome 
    • Rigorous hydration 
    81
    List the signs and symptoms of tumour lysis syndrome
    • Dysuria and oliguria 
  • Abdo pain  ## Footnote
  • N& V 
  • Muscle cramps 
  • Seizures 
  • Cardiac arrythmias 
  • Joint swelling/gout
    • 82
    What is lymphoma 
    • Type of cancer that affects lymphocytes inside the lymphatic system  ## Footnote
    83
    What is Hodgkins lymphoma 
    • The malignant proliferation of lymphoctes characterised by Reed-Strenberg cells ~ large cells that is binucleated 
    84
    List some risk factors for Hodgkins lymphoma 
    • HIV  ## Footnote
    • Immunosupresion 
    • Cigarette smoking 
    85
    List the signs and symptoms of Hodgkins lymphoma
    • Non tender unilateral lymphadenopathy ~ commonly cervical/supraclavicular nodes 
  • B symptoms ~ weight loss, pruritus, night sweats  ## Footnote
  • Alcohol induced painful lymphadenopathy 
    • 86
    What is the staging of Hodgkin lymphoma Ann Arbor staging syndrome
    • Stage I – involvement of a single nodal group
    • Stage II – involvement of two or more nodal groups on the same side of the diaphragm
    • Stage III – involvement of nodal groups on both sides of the diaphragm - above and below
    • Stage IV – disseminated disease with involvement of extralymphatic organs (eg. the bones or lung)
    • A/B refers to whether they have B symptoms or not like night sweats or fever 
    87
    Outline the investigations for Hodgkins lymphoma 
    • Lymph node biopsy ~ diagnostic and will show Reed sternberg cells  ## Footnote
    88
    Outline the management of Hodgkins lymphoma 
    Chemotherapy  ## Footnote
  • BEACOPP ~ bleomycin, etoposide, doxorucibin, cyclophosphamide, vincristine, procarbazine, prednisalone

    • Radiotherapy
    • Chemoradiotherapy 
    • Haemopoietic cell transplantation 
    89
    What are the subtypes of Hodgkins lymphoma 
    • Nuclear sclerosing ~ good prognosis  ## Footnote
    • Lymphocyte predominate ~ best prognosis
    • Lymphocyte depleted ~ worst prognosis 
    90
    List some complications of Hodgkins lymphoma 
    • Metastasis to breast and lungs
    91
    What is non Hodgkins lymphoma 
    • Includes all the lymphomas without Reed-Sternberg cells 
    92
    List some risk factors for non Hodgkins lymphoma 
    • HIV  ## Footnote
    • H.Pylori 
    • Hep B or C 
    • Exposure to pesticides 
    • Exposure to trichloroethylene 
    • FHx
    • Immunodeficiency states 
    93
    List some signs and symptoms of non Hodgkins lymphoma
    • Painless symmetrical lympadenopathy 
  • B symptoms ~ fever, night sweats, weight loss
  • Extra nodal diseases
  • Hepatomegaly + splenomegaly 
    • 94
    Outline the investigations for Non hodgkins lymphoma 
    • Excisional node biopsy ~ gold standard  ## Footnote
    • Blood film ~ nucleated red cells and left shift (early WBC precursors) 
    • Bone marrow biopsy - atypical lymphoid cells and irregular nucleus and high mitotic rate 
    • CT CAP and PET to stage 
    95
    Outline the management of non Hodgkin lymphoma 
    Depends on the specific subtype  ## Footnote
  • Chemotherapy R-CHOP-21 ~ rituximab, cyclophosphamide, doxorucibin, vincristine and prednisalone for 21 days 
  • Radiotherapy 
  • All pts recieve flu/pneumococcal vaccine 
  • Neutropenic pts ~ abx prophylaxis 
    • 96
    What are the 3 main types fo non Hodgkins lymphoma 
    • Diffuse large B cell lymphoma ~ associated with Hep C
    • Burkitts lymphoma ~ associated with EBV, malaria and HIV, painless lump in older 65's
    • MALT lymphoma ~ associated with H pylori 
    97
    Outline gastric MALT lymphoma 
    • Arises from B lymphocytes in marginal zone  ## Footnote
    • Has good prognosis 
    • Signs ~ abdo pain, N& V, anaemia, paraproteinaemia, extensive lymphocytes found on biopsy of mass 
    • Managed by eradicating H pylori 
    98
    Outline Burkitts lymphoma 
    • 2 types ~ endemic (african) and sporadic  ## Footnote
    • HIV is assocated with the sporadic form 
    • EBV (herpes virus 4) associated with endemic form 
    • Diagnosed by starry sky appearance, nucleated RBC, left shift
    • Management ~ chemotherapy
    99
    List some complications of non Hodgkins lymphoma 
    • Bone marrow infiltration  ## Footnote
    • Metastasis 
    • Spinal cord compression 
    • Side effects of chemo 
    100
    What is myeloma 
    • Haematological malignancy where there is clonal proliferation of plasma cells - plasma cell dyscrasia  ## Footnote
    101
    Briefly outline the pathophysiology of multiple myeloma
    • Abnormal proliferation of plasma cells which secrete monoclonal antibodies or paraprotein and M proteins into the serum adn urine 
  • Deficiency of functional antibodies - causes relative hypogammaglobunaemia 
    • 102
    List some risk factors for multiple myeloma 
    • Old age  ## Footnote
    • Black african ethinicty
    • FHx of haematological malignancy 
    • Obesity 
    • MGUS
    103
    List the signs and symptoms of multiple myeloma 
    CRAB HAIB
    • HyperCalcaemia  ## Footnote
    • Anaemia 
    • Bone pathology ~ back pain 
    • Hyperviscosity 
    • Amyloidosis 
    • Infection 
    • Bleeding + bruising 
    104
    Outline the investigations for multiple myeloma 
    • FBC ~ normocytic anaemia, thrombocytopenia, leukopenia  ## Footnote E's ~ raised urea, creatinine, calcium, normal/high phosphate and normal alkaline phosphate 
    • ESR 
    • Blood film ~ rouleaux formation 
    • Serum or urine protein electrophoresis ~ raised IgG/IgA paraprotein or raised Bence Jones protein - diagnostic 
    • Serum free light chain assay 
    • Bone marrow aspirate and biopsy ~ diagnostic 
    • Whole body MRI 
    • Skeletal survey 
    • Skull x-ray ~ raindrop skull 
    105
    Outline the management of multiple myeloma
    • Conservative management ~ regular follow up unless signs of active disease
    • Induction therapy ~ bortezomib, thalidomide, dexamethasone 
  • Followed by high dose melphalan as a conditioning regimen  ## Footnote
  • Erythropoietin for anaemia
  • After completion of treatment - pt monitered every 3 months with bloos tests and electrophoresis to check for relapse 
  • If relapse occurs ~ bortezomib monotherapy 
  • Myeloma bone disease ~ bisphosphonates and radiotherapy 
    • 106
    What are the X-ray findings in a pt with myeloma 
    • Well defined lytic lesions  ## Footnote
    • Abnormal fractures 
    • Pepper pot skull ~ multiple lytic lesion in the skull
    107
    What is leucocytosis 
    • Increase in the number of WBCs
    108
    What are the 5 types of leucocytosis 
    • Neutrophilia  ## Footnote
    • Basophilia 
    109
    List some causes of neutrophilia 
    • Bacterial infection  ## Footnote
    • Drug 
    • Pregnancy 
    • Stress
    • Smoking
    110
    List some causes of monocytosis
    • Chemo/radiotherapy 
  • Chronic infections 
    • 111
    List some causes of lymphocytosis 
    • Acute viral infection
    • Chronic infections
    • Leukaemias
    • Lymphomas 
    112
    List some causes of eosinophilia
    • Drug reactions
    • Allergies 
  • Parasitic infection  ## Footnote
  • Malignant disease 
  • Adrenall insufficiency 
    • 113
    List some causes of basophilia 
    • Myeloproliferative disease  ## Footnote
    • IgE mediated hypersensitivity reaction 
    • Inflammatory disorders
    114
    List some signs and symptoms of leucocytosis 
    • Fatigue  ## Footnote
    • Dyspnoea 
    • Splenomegaly 
    115
    What are the investigations for leucocytosis
    • FBC ~ increased WCC
    • Blood film 
  • Bone marrow biopsy 
    • 116
    Outline the management of leucocytosis 
    • Antibiotics if infection  ## Footnote
    • Medication to reduce stress or anxiety 
    • Anti inflammatory medication 
    • Inhalers for asthma 
    • IV fludis 
    • Leukapheresis ~ procedure to quicklt reduce WCC 
    • Cancer treatment 
    117
    What is neutropenia 
    • Low neutrophil count <
    118
    What is agranulocytosis 
    • Neutropenia with depleted basophils and eosinophils 
    119
    List some causes of neutropenia 
    • Severe sepsis  ## Footnote
    • Drugs 
    • Infiltration of the bone marrow
    • Haematological malignancies ~ myelodysplastic malignancies and aplastic anaemia 
    • Hypersplenism 
    • SLE 
    • Radio/chemotherapy 
    • B12, iron, folate deficiencies 
    120
    List some signs and symptoms of neutropenia 
    Often are just symptoms of an infection  ## Footnote
  • Fatigue 
  • Sore throat 
  • Swollen lymph nodes 
  • Pain, swelling or rash at affected site 
  • Diarrhoea
    • 121
    What are the investigations for neutropenia 
    • FBC
    • Find the cause ~ cultures, lumbar puncture, imaging etc 
    122
    What are the types of neutropenia 
    • Mild ~ 1-1.5 x 109
    • Moderate ~ 0.5-1 x 109
    • Severe ~ <
    123
    Whats the normal neutrophil count 
    • 2.0-7.5 x 109
    124
    Outline the management of neutropenia 
    • Use granulocyte colony-stimulating factor ~ stimus the production of neutrophil in bone marrow  ## Footnote
    • If febrile ~ quinolone with co amoxiclav 
    • Infection control ~ put the pt in a side room and use PPE
    125
    What is leukaemoid reaction 
    • Increase in WBC > ## Footnote
    126
    List some causes of leukamoid reaction 
    • Severe illness
    • Burns  ## Footnote
    • Haemorrhage 
    • Malignancy 
    • Intoxication ~ ethylene glycol 
    127
    List some signs and symptoms of leukamoid reactions 
      ## Footnote
  • Weakness
    • 128
    What are the investigations for leukamoid reaction 
    • Excluce leukaemia  ## Footnote
    • Blood film ~ increased neutrophil precursors, cytoplasmic toxic granulation, Dohle bodies
    • Bone marrow aspiration/biopsy 
    129
    How do you treat leukamoid reaction 
    • Treat underlying cause 
    130
    What is pancytopenia 
    • Combination of anaemia, thrombocytopenia and leukopenia (RBC, platelets and WBC)
    131
    What can cause pancytopenia 
    • Decreased marrow haematopoetic function  ## Footnote
    132
    List some signs and symptoms of pancytopaenia 
    • RBC ~ lethargy, pale skin, other symptoms of anaemia
    • WBC ~ fevers, infection 
    133
    Outline the investigations for pancytopaenia 
    • FBC ~ decreased WBC, RBC and platelets  ## Footnote
    • Bone marrow biopsy 
    134
    Outline the management of pancytopaenia 
    • Red cell transfusion  ## Footnote
    • Platelets ~ granulocyte colont stimulating factors
    135
    What is neutropaenic sepsis
    • Defined as a neutrophil count <
    0.5x109  ## Footnote
  • Symptoms and signs of sepsis 
  • Medical emergency!
    • 136
    What is the main cause of neutropaenic sepsis 
    • Chemotherapy ~ mainly 7-14 days after
    137
    What pathogens mainly cause neutropaenic sepsis 
    • Gram -ve ~ E.coli, P.aeruginosa, Klebsiella  ## Footnote
    138
    List some signs and symptoms of neutropaenic sepsis 
    • Often asymptomatic as they dont have enough WBC to mount a response but may have:
    • Tachycardia  ## Footnote
    • Fever ~ may not have this 
    • Sore throat
    • Immunosupressed
    139
    List some medications that can cause neutropaenic sepsis 
    • Chemotherapeutic drugs  ## Footnote
    • Hydroxychloroquine 
    • Methotrexate
    • Sulfasalazine 
    • Quinine 
    • Infliximab 
    • Azathioprine + allopurinol
    140
    What are the investigations for neutropaenic sepsis 
    • FBC ~ WCC decreased  ## Footnote
    • Blood cultures
    • CXR 
    • Serology and PCR 
    • Sputum, urine and stool samples 
    • Swabs 
    141
    Outline the management of neutropaenic sepsis 
    • IV broad spectrum abx ~ piperacillin with tazobactam  ## Footnote
    • Sepsis 6 ~ IV fluids, oxygen, blood cultures, lactate measures, urine output 
    • If they are low risk ~ oral abx 
    • Daily measures of fever and baseline bloods until the patient is apyrexial and neutrophil count is > 0.5x109
    142
    What is haemophilia 
    • X linked recesive inheritied bleeding disorders 
    143
    What causes haemopholia A
    • Deficiency in Factor VIII
    144
    What causes haemophilia B
    • Lack of factor IX
    145
    List some signs and symptoms of haemophilia 
    • Spontaneous deep and severe bleeding into soft tissues, joints and muscles  ## Footnote
    • Bruisings 
    • Intercranial haemorrhage 
    • Cord bleeding 
    • Other bleeding in unusual sites like gums, GIT etc
    • Failure to walk in toddles due to bleeding into the joints 
    146
    What are the investigations for haemophilia 
    • Factor VIII or IX assay  ## Footnote
    • Prothrombin time is normal 
    • Genetic testing 
    147
    Outline the management of haemophilia 
    • Minor bleeding ~ desmopressin 
    148
    What is the emergency management of haemophilia 
    • Infusion of the affected factor ~ VIII or IX  ## Footnote
    • Antifibrinolytics 
    149
    What is Von Willebrands disease 
    • Inherted bleeding disorder characterised by a reduced quantity or function of von Willebrand factor  ## Footnote
    150
    What are the 3 types of Von Willebrands disease 
    • Type 1 ~ partial reduction in vWF ~ most common  ## Footnote
    • Type 3 ~ total lack of VWF ~ most severe
    151
    What causes Von Willebrands disease 
    • Most are due to an autosomal dominant inheritance  ## Footnote
    152
    Briefly outline the pathophysiology of Von Willebrand disease
    • This protein normally links platelets to the exposed endothelium and stabilises clotting factor VIII 
  • Dysfunction or deficiency leads to an increased risk of bleeding
    • 153
    List the signs and symptoms of Von Willebrands disease 
    • Hx of unusually easy, prolonged or heavy bleeding  ## Footnote
    • Epistaxis 
    • Excess or prolonged bleeding from minor wounds or post operatively 
    • Easy bruising
    • Epistaxis 
    • GI bleeding
    154
    Outline the investigations for Won Willebrands disease 
    • Clotting tests ~ normal PT and TT  ## Footnote
    • Platelet count is normal 
    • vWF level and activity assay 
    • Factor VIII activity is normal but decreased becasue its bound to VWF
    155
    Outline the management of Von Willebrands disease 
    • Mild bleeding ~ desmopressin (first line)
    • Mild bleeding + heavy mensturation ~ tranxamic acid or mefenemic acid, COCP or mirena coil  ## Footnote
    156
    What are thrombophilias 
    • Conditions that predispose patients to develop blood clots due to dysregulation of the coagulation system 
    157
    What can cause inherited thrombophilias 
    • A deficiency in a natural anticoagulant ~ antiphospholipid syndrome, antithrombin III deficiency or Protein C or S deficency  ## Footnote
    158
    What is factor V leiden 
    • Mutation in factor V causes it become ressitant to inactivation by protein c 
    159
    List some signs and symptoms of thrombophilias
    • Recurrent venous clots 
  • Calf swelling ~ DVT  ## Footnote
  • Chest pain ~ PE
  • Breathlessness ~ PE
  • Hypotension ~ PE
  • Tachycardia and tachypnoe ~ PE
    • 160
    What is anti thrombin III deficiency 
    • Anti thrombin normally inihibits factor IIa, Xa, IXa and XIa
    • Deficiency increases the risks of thrombosis  ## Footnote
    • Heterzygosity increases the risk fo VTE by 50 folds
    161
    What is protein C deficiency 
    • Protein C inactivates clotting factors V and VIII
    • Inactivating mutation in the protein C increass the risk of thrombosis 
    162
    What is protein S deficiency 
    • Protein S is a vitamin k dependent co factor for the anticoagulation activity of protein C 
    163
    List some risk factors for VTE
    • Age 
  • Pregnancy  ## Footnote
  • Antiphopsholipid syndrome 
  • Previous PE or DVT 
  • Being bed bound over 5 days 
  • Dehydration 
  • Recent surgery 
  • COCP+HRT 
  • Smoking 
  • Long haul air travel 
  • Obesity
    • 164
    How does increasing age increase risk of VTE
    • As you increase in age, the levels of activated factor VII, IX and X increases and well as increased levels of factor XII, fibrinogen and D dimer which all increase risk of thrombosis
    165
    How does pregnancy/post partum increase risk of VTE
    • Pregnancy causes a fall in protein S and increase in fibrinogen, factor VIII and vWF which results in activated protein C resistance - clots can form 
    166
    How does malignancy increase risk of VTE
    • The pt is in a prothrombic state and there is greater activation of the coagulation system due to TF expression and fibrinolytic activity 
  • Malignant cells will also release cytokines and will interact with endothelial cells and platelets
    • 167
    What is antiphospholipid syndrome
    • An autoimmune disorder characterised by arterial and venous thrombosis, adverse pregnancy outcomes and raised levels of antiphospholipid antibodies 
    168
    List the main features of antiphospholipid syndrome 
    • Clots ~ usually venous thromboembolisms rather than arterial  ## Footnote
    • Obstetric loss ~ recurrent miscarriages or premature births
    • Thrombocytopenia 
    • Cardiac valvular disease 
    169
    Outline the investigations for antiphospholipid syndrome
    One or more of the following positive blood tests are needed on 2 occasions, 12 weeks apart to diagnose it:
    • Anti-cardiolipin AB's
    • Anti-beta2-GPI AB's
    • Positive lupus anticoagulant assay
    170
    Outline the management of anti phospholipid syndrome 
    • Primary prophylaxis ~ low dose aspirin  ## Footnote
    • Arterial thrombosis is treated with lifelong warfarin with target INR 2-3
    • Remember, warfarin is teratogenic and in pregnancy use alternative anticoagulation like low molecular heparin 
    171
    What are the VTE prophlaxis treatment available 
    • If a pt is at increased risk of VTE they should recieve low molecular weight heparin such as enoxaparin  ## Footnote
    • Venous VTE ~ avoid oestrogen containing contraception, HRT and long period of immobility 
    • Arterial ~ control CV risk factors
    172
    What is TTP
    • A condition where tiny thrombi develop throughout the small vessels and use up the platelet supply
    • It is characterised by microangiopathic haemolytic anaemis and thrombocytopenic purpura 
    173
    List some causes of TTP
    • Idiopathic 
  • Pregnancy  ## Footnote
  • Drugs 
  • Post infection 
  • Tumours 
  • SLE
    • 174
    Outline the pathphysiology of TTP
    • Dysfunction in the ADAMTS13 protein
    • This protein normally inactivates vWF and reduces platelet adhesion and clot formation 
  • Deficiency means overactivation of vWF - clots form - RBC breakdown and causes anaemia 
    • 175
    List some signs and symptoms of TTP
    • Neurological abnoramlity - seizures
    • Fever 
  • Renal failure  ## Footnote
  • MAHA on blood film
  • PT and APTT are normal 
    • 176
    Outline the investigations for TTP
    • Platelets ~ low 
  • Hb ~ low  ## Footnote
  • LDH/troponin ~ high 
  • Urea and creatinine ~ high 
  • Blood film ~ schistocytes
    • 177
    Outline the management of TTP
    • Plasma exchange ~ gold standard 
  • Steroids  ## Footnote
    • 178
    What is INR
    • A measure of how long it takes blood to form a clot
    • It has to be monitored in pts who are on anticoagulants
    179
    What is the target INR for patients on anticoagulants
    • 2-3
    180
    What is a high INR
    • It means blood too thin which means there is a risk of excessive bleeding 
  • An INR > ## Footnote
    • 181
    What is a low INR
    • It means blood is too thick which means the risk of clotting/thrombosis 
  • INR < ## Footnote
    • 182
    List some causes of high INR
    • Overdose of anticoagulant medication 
  • Drug interactions  ## Footnote
  • Increase in alcohol consumption 
  • Low vitamin K 
  • Infection 
    • 183
    List some signs and symptoms of high INR
    • Any evidence of bleeding 
  • Overt blood loss
  • Bruising 
    • 184
    Outline the investigations for high INR History 

    • Dosing history of anticoagulants  ## Footnote
    • Change in diet/lifestyle/medications 
    • History of any falls/injuries 
    • History of blood loss
    Bloods
    • FBC ~ check for signs of anaemia and infection 
    • Clotting screen ~ check of any other abnormalities 

    • Do a CT head if you are worried by intercranial haemorrhage 

    185
    Outline the management of high INR greater than 8 if there is major bleeding 
    • Stop anticoagulants 
    186
    Outline the management of high INR greater than 8 if there is minor bleeding 
    • Stop anticoagulation  ## Footnote
    • Repeated INR after 24hrs and see if they need further vitamin K
    • Restart warfarin when INR is below 5
    187
    Outline the management of high INR >
    8 with no bleeding
    • Stop anticoagulants  ## Footnote
    • Repeat INR after 24 hours
    188
    Outline the management of high INR >
    5 and no bleeding
    • Withold 1-2 doses of anticoagulants 
    189
    When can you restart warfarin 
    • When the INR is less than 5 
    190
    What is DIC
    • It is the inappropriate activation of clotting cascade resulting in thrombus formation and depletion of clotting factors and platelets
    • It is a microangiopathic haemolytic anaemia
    191
    List some causes of DIC
    • Sepsis 
  • Trauma/burns  ## Footnote
  • Infections 
  • Vascular conditions
  • Obstetrics complications 
    • 192
    Outline the pathophysiology of DIC
    • TF is on many cells but not normally in contact with general circulation ~ it is exposed after tissue damage 
  • When activated, TF binds coagulation factors triggering after extrinsic pathways and instrinsic pathways 
    • 193
    List the signs and symptoms of DIC
    • Excessive bleeding 
  • Fever
  • Hypotension  ## Footnote
  • Petechiae
    • 194
    Outline the investigations for DIC Blood tests:
    • FBC ~ shows thrombocytopenia
    • Decreased fibrinogen 
  • Increased prothrombin time and APTT
  • Raised D dimer

    • Blood film shows schistocytes (broken RBCs) due to microangiopathic haemolytic anaemia
    195
    Outline the management of DIC 
    • Traet the underlying cause
    • Cyroprecipitate or FFP first
    • Blood products transfusion ~ platelets, FFP to replace coagulation factors and cyroprecipitate to replace fibrinogen 
    196
    What is massive blood loss
    • Sudden and continuing blood loss of greater than 2 litres
    197
    What 4 products can be readily transfused 
    • RBC's
    • FFP  ## Footnote
    198
    What blood group is the universal donor for packed RBC's
    • O
    199
    What are the indications for packed red cell transfusions
    • Symptomatic/chronic anaemia 
  • Major haemorrhage 
    • 200
    What is the universal donor for FFP
    • AB
    201
    What is in FFP
    • Clotting factors 
  • Antibodies  ## Footnote
    • 202
    What are the indications for FFP
    • Bleeding due to multi factor deficiencies 
  • Raised PT/APTT
  • Prophylactic correcting clotting factors before high risk surgery 
    • 203
    What is the main contraindication for FFP
    • Dont use a volume expander
    204
    What is the indication for platelets 
    • Thrombocytopenia 
    205
    List the contraindications for platelet transfusions
    • Heparin induced thrombocytopenia 
  • Chronic bone marrow failure  ## Footnote
  • Autoimmune thrombocytopenia 
    • 206
    What is in cyroprecipitate 
    • Contains factor VIII, vWF and fibrinogen 
    207
    List the indications for cyroprecipitate 
    • Clinically significant haemorrhage  ## Footnote
    • Allows large amount of clotting factor to be administered in a small volume 
    • Low fibrinogen 
    208
    What are the indications for prothrombin complex concentrate 
    • Emergency reversal of anticoagulanttion in pts with severe bleeding 
    209
    What are cell saver devices 
    • They collect the pts own blood lost during surgery and reinfuse ir  ## Footnote
    210
    What is the main advantage of cell saver devices 
    • Avoids the use of infusing donor blod therefore reduces the risk of blood bourne infection
    211
    What are the 2 types of transfusion reactions 
    • Acute
    • Chronic
    212
    What are the types of acute transfusion reactions 
    • Acute haemolytic  ## Footnote
    • Allergic 
    • Transfusion related acute lung injury 
    • Transfusion associated criculatory overload
    213
    Outline an allergic reaction to blood transfusion 
    • Cause ~ reaction to foreign components in transfusion  ## Footnote
    • Management ~ stop transfusion, saline adrenaline and oxygen if needed, antihistamine 
    214
    Outline acute haemolytic transfusion reaction to a blood transfusion 
    • Cause ~ incompatible blood given ang IgM mediated RBC destruction
    • Features ~ fever, hypotension, abdo/chest pain, agitation 
    215
    Outline febrile non haemolytic transfusion reaction to a blood transfusion 
    • Cause ~ antibodies to WBC HLA
    • Features ~ fevers and chills 
    216
    Outline transfusion related acute lung injury
    • Cause ~ increased vascular permeability caused by host neutrophils which are activated by donor blood 
  • Features ~ pulmonary oedema, ARDS, hypoxia, hypotension, fever, white out on CXR
  • Management ~ stop the transfusion, give saline and treat ARDs
    • 217
    Outline transfusion associated circulatory overload
    • Cause ~ fluid overload
    • Features ~ pulmonary oedema and hypertensive 
  • Management ~ slow the transfusion and give furosemide 
    • 218
    List some delayed reactions to blood tranfusions 
    • Delayed haemolytic transfusion reaction  ## Footnote
    • Post transfusion purpura 
    219
    Outline delayed haemolytic transfusion reaction 
    • Cause ~ exaggerated response to foreign antigen  ## Footnote
    • Management ~ fluids 
    220
    Outline transfusion associated graft-verses-host disease 
    • Cause ~ donot lymphocytes attacking recipient body 
    221
    Outline post transfusion purpura
    • Cause ~ immune response against platelets 
  • Features ~ bleeding  ## Footnote
    • 222
    How is iron overload treated 
    • Subcutaneous desferrioxamine 
    223
    Outline the Billingham criteria to diagnose graft vs host disease
    • Graft tissue contains immunologically functioning cells 
  • Recipient and donor are immunologically different  ## Footnote
    • 224
    What is polycythaemia 
    • Its an increase in haematocrit, red cell count and haemoglobin concentration  ## Footnote
    225
    What causes relative polycythaemia 
    • Falsely elevated haemoglobin secondary to a low plasm volume such as dehydration, excess diuretic use, diarrhoea etc
    226
    What is absolute polycythaemia 
    • Plasma volume is normal and red cell mass is raised 
    227
    List some causes of secondary polycythaemia 
    Its due to inappropriate rise in erythropoetin  ## Footnote
  • Altitude 
  • Obstructive sleep apnoea 
  • Excess erythropoeitin ~ cerebellar haemnagioma, hepatoma, uterien fibroids 
    • 228
    What is primary polycythaemia 
    • There is excessive and uncontrolled erythrocytosis that is independent to erythropoetin levels 
    229
    How do you differentiate between absolute and relative polycythaemia 
    • Use red cell mass studies  ## Footnote 35ml/kg and in women > 32ml/kg
    230
    List some signs and symptoms of polycythaemia 
    • Splenomegaly  ## Footnote
    • Low platelets 
    • Thrombosis ~ arterial and venous
    • Raised RBC 
    • Low WBC 
    • Erythromelalagia
    • Facial redness
    231
    What is polycythaemia rubra vera 
    • A myeloproliferative disorder caused by clonal proliferation of marrow stem cell leading to increase in red cell volume as well as overproduction of neutrophils and platelets
    232
    What causes polycythaemia rubra vera 
    • Mutation in JAK2 gene
    233
    List some risk factors for polycythaemia rubra vera
    • Budd-chiari syndrome 
  • Greater than 40 years old 
    • 234
    List the features of polycythaemia rubra vera 
    • Hyperviscosity  ## Footnote
    • Haemorrhage 
    • Plethoric appearance ~xs redness in eye conjucntiva 
    • May be accompanied with high neutrophil and platelets
    235
    Outline the investigations for polycythaemia 
    • FBC and blood film ~ raised haematocrit, neutrophils, basophils and platelets  ## Footnote
    • Serum ferritin 
    • Renal and liver function tests 
    • Vit B12 levels 
    • Bone marrow biopsy 
    236
    Outline the management of polycythaemia 
    • Venesection ~ first line  ## Footnote
    • Cytoreductive therapy if venesection doesnt work ~ 1st line is hydroxyurea and in younger patients its interferon 
    • Allopurinol for gout 
    237
    What is essential thrombocythaemia 
    • Chronic myeloproliferative disorder caused by dysregulated megakaryocytes proliferation causing an abnormally high platelet count >
    238
    What causes essential thrombocythaemia 
    • Due to JAK2 V617F mutation 
    239
    Why does essential thrombocythaemia increase the risk of bleeding 
    • All the excessive platelets will use up the free vWF which means not enough will be available at the site of injury 
    240
    List some risk factors for essential thrombocythaemia 
    • Female 
    241
    List some signs and symptoms of essential thrombocythaemia 
    • Thrombosis  ## Footnote
    • Splenomegaly 
    • Erythromelalgia ~ discoluration and pain in the extremities 
    • Hyposplenism 
    • Livedo reticularis 
    • Systemic features ~ fatigue, weight loss etc
    242
    Outline the investigations for essential thrombocythaemia 
    • FBC ~ increases platelet count  ## Footnote
    • Low iron 
    • Genetic testing for JAK2 V617F mutation
    243
    Outline the management of essential thrombocythaemia 
    • Hydroxyurea ~ reduce platelet count  ## Footnote
    • Low dose aspirin ~ reduce thrombotic risk 
    244
    What is anaemia of chronic disease
    • It is generally normocytic, normochromic anaemia 
  • Characterised by low Hb levels, decreased RBC production and altered iron metabolism 
    • 245
    List some causes of anaemia of chronic disease Due to chronic inflammation of an underlying disease:
    • Malignancy 
  • Chronic infections  ## Footnote
  • Autoimmune conditions 
  • Surgery 
    • 246
    Outline the pathophysiology of ACD
    • Chronic inflammation produces inflammatory cytokines like IL-6
    • Raised IL6 will stimulate the release of hepcidin from the liver which reduces iron absorption by reducing the activity of ferroportin - iorn channel 
  • This leads to decrease in Hb production  ## Footnote
    • 247
    Lists some signs and symptoms of ACD
    • Pallor 
  • Fatigue  ## Footnote
  • Headache/dizziness
    • 248
    Outline the investigations for ACD
    • FBC ~ low Hb and low MCV
    • Blood film ~ normocytic normochromic 
  • Serum iron studies ~ low serum iron, high ferritin and low transferritin saturation low TIBC  ## Footnote
    • 249
    Outline the management of ACD
    • Treat underlying cause 
  • IV iron therapy  ## Footnote
  • Erythropoietic agents 
    • 250
    What is haemochromatosis 
    • Autosomal recessive genetic condition resulting in iron overload
    • Caused by mutation in the HFE gene on both copies of chromosome 6 
    251
    List the signs anf symptoms of haemochromatosis 
    • Chronic tiredness  ## Footnote
    • Joint pain 
    • Swollen joints
    • Bronze skin pigmentation 
    • Testicular atrophy 
    • Erectile dysfunction 
    • Amenorrhoea 
    • Cognitive symptoms
    • Hepatomegaly 
    • Hypogonadotrophic hypogonadism due to iron deposits in the pituitary therefore cannot release gonadotrophins
    252
    Outline the investigations for haemochromatosis 
    • Bloods ~ deranged LFTs, raised serum ferritin and raised transferritin saturation, raised serum iron, decreased total iron binding capacity  ## Footnote
    • Liver biopsy ~ Perl stain confirms increased iron stores
    253
    Outline the management of haemochromatosis 
    • Venesection ~ remove some blood every week  ## Footnote
    • Avoid fruit juices 
    • Monitor serum ferritin and transferritin
    254
    List some complications of haemochromatosis 
    • Secondary diabetes  ## Footnote
    • Cardiomyopathy 
    • Hepatocellular carcinoma 
    • Hypothyroidism 
    • Chrondrocalcinosis ~ calcium pyrophosphate deposits in the joint 
    255
    What is HDN
    • An autommune condition where a rhesus negative mum becomes sensitised and develops antibodies against her rhesus positibe blood cells of her baby in utero 
    256
    List some events in which a RhD-ve mum can develop anti RhD ab's
    • Antepartum haemorrhage 
  • Abdominal trauma  ## Footnote
  • Invasive uterine procedure 
  • Ectopic pregnancy 
  • Intrauterine death 
  • Transfusion of RhD+ve blood 
  • Delivery 
    • 257
    Outline the pathophysiology of HDN
    • During a sensitisation event, the mothers blood mixes with the RhD+ve antigens from the foetus and develops IgG AB's agaisnt the RhD
    • So if the mother has a 2nd RhD+ve child, the anti Rhd IgG can cross the placenta and cause haemolysis of Rh+ve RBC's of the baby 
    258
    List some signs and symptoms of HDN
    • Hydrops foetalis ~ foetal oedema in 2 diff compartmetns seen on US
    • Yellow coloured amniotic fluid due to excess bilirubin 
  • Neonatal jaundice and kernicterus  ## Footnote
  • Hepatomegaly or splenomegaly
  • Heart failure 
    • 259
    Outline the invesitgations for HDN
    • Test maternal blood type 
  • Maternal serum RhD ab screen  ## Footnote
  • Kleihauer test   
    • 260
    Outline the management of HDN for the mothers 
    • Anti-D abs at 28 weeks and at birth of the RhD+ve baby  ## Footnote
    • Early delivery if needed 
    261
    Outline the management of HDN for the neonate 
    • Exchange transfusion to manage high bilirubin  ## Footnote
    262
    What is haemorrhagic disease of the newborn 
    • Vit K deficiency bleeding 
    263
    What casues haemorrhagic disease of the newborn 
    • Deficiencies in Vit K as it cannot cross the placenta 
    264
    What clotting factors require vit K for its production 
    • Factor II, VII, IX and X
    265
    What are the risk factors for haemorrhagic disease of the newborn 
    • Breast fed babies
    • Maternal use of anti epileptics
    266
    List some signs and symptoms of haemorrhagic disease of the newborn 
    • Easy bruising  ## Footnote
    • Internal bleeding 
    • Juandice
    267
    Outline the investigations for haemorrhagic disease of the newborn 
    • FBC  ## Footnote
    • CXR or US for inter throacic bleed 
    • CT or MRI for intercranial bleed
    268
    Outline the management of haemorrhagic disease of the neonate
    • Vit K supplement ~ subcut offered routinely at birth 
  • FFP if severe bleeding
    • 269
    What is the main complication of haemorrhagic disease of the newborn 
    • Haemorrhagic shock 
    270
    What is immune thrombocytopenia 
    • Autommune condition characterised by the reduction in the number of circulation platelets 
    271
    List some signs and symptoms of immune thrombocytopenia 
    • Bruising  ## Footnote
    • Mucocutaneous bleeding
    • Blood in urine or stool 
    • Bleeding
    • History of recent viral infection
    272
    Outline the investigation for immune thromboytopenia 
    • FBC ~ isolated thrombocytopenia  ## Footnote
    273
    Outline the management of immune thrombocytopenia 
    • Usually self limiting ~ wait and watch  ## Footnote
    • IV steroids 
    • IV immunoglobulins - second line
    • Platelet transfusion ~ avoided unless life threatening bleeding
    • Splenectomy 
    274
    What is the definition of a major bleed 
    • intracranial, retro-peritoneal, intraspinal, intra-ocular, pericardial or intramuscular bleeding with compartment syndrome
    275
    What is the reversal agent for heparin 
    • Protamine sulphate 
    276
    What is the reversal agent for Warfarin 
    • Vit K and prothrombin complex concentrate
    • FFP only to be used if PCC is unavailable 
    277
    What is the emergency management of a major bleed
    • Stop the anti coag 
  • Correct haemodynamics  ## Footnote
    • 278
    What is sideroblastic anaemia
    • The body produced enough iron but is unable to put it into haemoglobin
    279
    List some causes of normocytic anaemia 
    • anaemia of chronic disease
    • chronic kidney disease
    • aplastic anaemia
    • haemolytic anaemia
    • acute blood loss
    280
    How do Reed Sternberg cells look
    • Large cells that are multinucleated or have a bilobed nucleus 
  • Prominent eosinophilic inclusion like nuclei - owl's eye appearance
    • 281
    What is the reversal agent for apixaban and rivaroxaban 
    • IV andexanet alfa 
    282
    What bloods would you do for myeloma
    • FBC ~ anaemia 
  • Peripheral blood film ~ rouleaux formation  ## Footnote E's ~ high urea and creatinine suggesting renal failure 
  • Bone profile ~ hypercalacaemia 
    • 283
    What will protein electrophoresis show 
    • Raised monoclonal proteins in the serum 
    284
    What will bone marrow aspiration show 
    • Significantly riased plasma cells
    285
    What is the long term management of sickle cell disease 
    • Hydroxyurea to increase HbF levels 
    286
    How does acute chest syndrome present 
    • Dyspnoea  ## Footnote
    • Cough 
    • Hypoxia 
    • New pulmonary infiltrated on CXR
    287
    What is the complication of polycythaema rubra vera
    • AML
    288
    What is the reversal agent for dabigatran
    • Idarucizumab
    289
    What conditions have target cells 
    • Sickle cell
    • Thalassaemia  ## Footnote
    • Liver disease
    290
    What conditions have tear drop poikilocytes 
    • Myelofibrosis 
    291
    What conditions have spherocytes 
    • Hereditary spherocytosis 
    292
    What conditions have basophillic stippling
    • Lead poisoning 
  • Thalassaemia  ## Footnote
  • Myelodysplasia
    • 293
    What conditions have Howell Jolly bodies 
    • Hyposplenism
    294
    What conditions have Heinz bodies 
    • G6PD deficiency 
    295
    What conditions have schistocytes - helmet cells 
    • Intravascular haemolysis  ## Footnote
    • DIC
    296
    What conditions have pencil poikilocytes 
    • Iron deficiency anaemia
    297
    "What is this 
    "
    • Spherocytes 
    298
    "What is this 
    "
    • Tear drop Poikilocytes
    299
    "What is this
    "
    • Target cells
    300
    "What is this 
    "
    • Heinz bodies 
    301
    "What is this 
    "
    • Howell Jolly bodies 
    302
    "What is this 
    "
    • Basophillic stippling
    303
    "What is this 
    "
    • Pencil poikilocytes
    304
    "What is this 
    "
    • Schistocytes - helmet cells 
    305
    How can you differentiate between haemophilia and VWB disease 
    • Haemophilia ~ bleeding into joints more common, also very very rare in women cos its X linked  ## Footnote
    • VWD ~ mennorhagia and GI bleeding more common
    306
    What symptoms indicate Richters transformation
    • Lymph node swelling 
  • Fever withoht infection  ## Footnote
  • Night sweats 
  • Nausea 
  • Abdo pain
    • 307
    How would aplastic crisis present 
    • Caused by infection with parvovirus  ## Footnote
    • Reduced reticulocyte count
    308
    How would sequestration crisis present 
    • Increased reticulocyte count  ## Footnote
    • Abdominal pain 
    309
    List the signs and symptoms of haemolytic anaemia 
    • Fatigue  ## Footnote
    • Juandice
    • Splenomegaly 
    • Dark urine 
    • Gallstones 
    • Leg ulcers 
    • SOB
    • Palpitations
    310
    What will a Hb electrophoresis show in beta thalassaemia major 
    • HbA2 and HbF are produced  ## Footnote
    311
    What will Hb electrophoresis show in sickle cell anaemia
    • HbS produced
    312
    What is the bleeding pattern of haemophilia 
    • Deep bleeding like - joint bleeding, muscular haematomas, big bruises etc 
    313
    What is the bleeding pattern in VWB disease Platelet pattern 

    • Cutaneous petechiae and purpura  ## Footnote
    • Mucosal bleeding - GI and mennorhagia 
    314
    Who are pts with low risk ETP
    • Age less than 40 AND
    • Platelet count <
    1500
  • No history of thrombosis or haemorrhage 
    • 315
    How are pts with low risk ETP treated
    • Aspirin alone 
    316
    Which pts are high risk with ETP
    • Aged 
    over 60 OR  ## Footnote 1500
  • Previous history of thrombosis or haemorrhage 
  • Diabetes or hypertension 
    • 317
    How are high risk ETP pts treated
    • Hydroxycarbamide/hydroxyurea AND aspirin
    318
    How are intermmediate risk ETP pts treated
    • Hydroxycarbamide and aspirin or just aspirin alone
    319
    How can you distinguish between IPT and TTP 
    • ITP will present with isolated thrombocytopenia and and some signs of bleeding/bruising like petechiae and purpura  ## Footnote
    320
    What is haemolytic anaemia 
    • Increased peripheral destruction of RBC's
    321
    What are the categories of haemolytic anaemia 
    • Inherited vs acquired  ## Footnote
    • Extravascular vs intravascular
    322
    Give some examples of inherited haemolytic conditions 
    • Sickle cell disease  ## Footnote
    • G6PD deficiency 
    323
    Give some examples of acquired haemolytic conditiosn 
    • Paroxysmal nocturnal haemoglobinuria  ## Footnote
    • TTP
    • DIC
    • Warm and cold autoimmune 
    324
    What is involved in a haemolysis screen 
    • FBC  ## Footnote
    • Reticulocytes - high 
    • LDH - increased 
    • Bilirubin - increase in uncongugated 
    • Haptoglobin - decreased as it takes up free bilirubin 
    • DAT - positive in autoimmune cases 
    325
    What test results will suggest intravascular haemolysis 
    • Increased uncongujated bilirubin  ## Footnote
    • Increased reticulocytes 
    • Decreased haptoglobin 
    • Haemoglobinuria 
    • Haemoglobinaemia 
    • Hemosidernuria 
    326
    What tests results suggest extravascular haemolysis 
    • Increased unconjugated bilirubin  ## Footnote
    • Increased reticulocyte
    • Everything else normal
    327
    What Ig is responsible for warm AIHA
    • IgG
    328
    What Ig is responsible for cold AIHA
    • IgM
    329
    What is the investigation for AIHA
    • Direct antiglobulin test/Coombs test 
    330
    How is warm AIHA treated 
    • Steroids  ## Footnote
    • Splenectomy
    331
    How is cold AIHA treated 
    • Keep warm  ## Footnote
    • Chemotherapy if lymphoproliferative disorder
    332
    What is MAHA
    • Microangiopathic haemolytic anaemia 
  • Mechanical destruction of RBC which show as fragments on blood film - schistocytes  ## Footnote
    • 333
    What is the pentad of TTP
    • Fever 
  • Renal failure  ## Footnote
  • Thrombocytopenia 
  • MAHA on blood film
    • 334
    What bloods suggest TTP
    • Low platelets 
  • Low Hb  ## Footnote
  • High LDH 
  • High creatinine +/- positive troponin
    • 335
    What is the pathophysiology of TTP
    • ADAMTS13 normally proteolyses vWF to inactivate it 
  • However in TTP there is reduction in ADAMTS14 which causes platelet aggregation and tiny thrombi forms using up platelets
    • 336
    What is the treatment for TTP
    • Plasma exchange 
  • High dose steroids  ## Footnote
  • Aspirin and prophylactic LMWH when platelets recover
    • 337
    What causes sickle cell disease
    • Point mutation in the beta globin gene on chromosome 11 
    338
    What is the key investigation for sickle cell disease 
    • Gel electrophoresis 
    339
    What is the emergency management of sickle cell 
    • Pain relief  ## Footnote
    • Antibiotics 
    • Blood transfusions 
    340
    What is the long term management of sickle cell
    • Hydroxycarbamide
    • Pneumoccocal vaccine every 5 years
    341
    Where are the sites of bleeding in VWD 
    • Skin 
    342
    What is petechiae 
    • Pin point non blanching spots that are less than 2mm and affect the skin and mucous membrane
    343
    List the bleeding features in VWD
    • Petechiae 
  • Small superficial bruises  ## Footnote
  • Mild and immediate bleeding after surgery or trauma 
    • 344
    Where are the sites of bleeding in coagulation factor deficiencies 
    • Deep in soft tissue like muscle nad joint 
    345
    List the bleeding features in coagulation factor deficiencies 
    • No petechiae  ## Footnote
    • Haemoarthrosis and muscle bleeding 
    • Severe but delayed bleeding after surgery or trauma 
    346
    What are the important components of blood clots 
    • Fibrin mesh  ## Footnote
    347
    What does an abnormal prothrombin time suggest
    • Factor VII deficiency 
    348
    What does an abnormal APTT suggest 
    • Deficiency in:
    • Factor VIII, IX or XI 
    349
    What does an abnormal PT and APTT suggest 
    • Deficiency in factor II, V or X
    350
    What does an abnormal thrombin time suggest 
    • Deficiency in fibrinogen 
    351
    What is PT testing 
    • Test of extrinsic coagulation pathway 
    352
    What is APTT testing
    • Test of intrinsic coagulation pathway 
    353
    What reagenets do you add to test clotting time 
    • Phospholipids - co factors  ## Footnote
    • Calcium 
    354
    What are some causes of cytopenias
    • Reduced cell production - B12, folate, iron deficiency 
  • Excess loss of cells or destruction 
    • 355
    What are the 2 classifications of BMF
    • Inherited 
  • Acquired 
    • 356
    What is aplastic anaemia 
    • The body stops producing all type of blood cells 
    357
    What causes aplastic anaemia
    • Radiation 
  • High dose chemo  ## Footnote
  • Viral - hepatitis 
  • Idiopathic - most cases
    • 358
    How is aplastic anaemia diagnosed 
    • FBC  ## Footnote
    • Retics 
    • Viral studies 
    • LFTs
    359
    What are the complications of aplastic anaemia 
    • Risk of infection  ## Footnote
    • Iron overload 
    • Reduced QoL
    • Evolve to paroxysmal nocturnal haemoglobinuria 
    • Transform to acute leukaemia 
    360
    What are the treatment options for aplastic anaemia 
    • Supportive - blood products, antibiotics
    • Immunosuppressants
    • Growth factors  ## Footnote
    361
    What is Fanconi anaemia 
    • Rare genetic disorder affecting RBCs, WBCs and platelets
    • The mutation causes high frequency of chromosome breakage 
    362
    What are the signs of Fanconi anaemia
    • Skin - cafe au lait 
  • Skeletal - absent thumbs  ## Footnote
    • 363
    How does Falconi anaemia present 
    • Difficult to diagnose  ## Footnote
    • Presents between 5-10 
    364
    How does Falconi anaemia progress
    • Bone marrow failure in 90%
    • Acute leukaemia 
  • Solid tumours 
    • 365
    What is the treatment for Falconi anaemia
    • Bone marrow transplant - definitive
    • Prior to BMT - supportive treatment, steroids and androgens 
    366
    How is aplastic anaemia present 
    • Hb < ## Footnote 50 
    • Neutrophils < 1.5
    367
    What is transient red cell aplasia 
    • Infection with parvovirus B19 infects and destroys erythroid precurosr cells  ## Footnote
    • Normally nothing to worry about in normal people but can be lfie threatening in those with underlying chronic anaemia 
    368
    How do you diagnose parvovirus induced aplasia
    • Parvovirus B19 serology testing 
  • PCR
    • 369
    What is the treatment for parvovirus induced aplasia 
    • Supportive - isolation  ## Footnote
    • Keep away from pregnant women 
    370
    What is the emergency management of neutropenic sepsis
    • IV broad spectrum abx immediately - IV piperacillin with tazobactam normally 
    371
    What is thrombocytopenia characterised by 
    • Purpura  ## Footnote
    • Overt bleeding - mennorhagia, epsitaxis GI bleed etc
    372
    What causes thrombocytopenia
    • Inherited 
  • Acquired  ## Footnote
  • Micorangiopathic disease 
  • Septicaemia 
    • 373
    What is ITP and its treatment
    • Happen after an infection and often resolves 
  • Can be treated with oral steroids  ## Footnote
  • Serious bleeding - give platelets and maybe RBC transfusion
  • Chronic cases - immunosuppressants and steroid sparing agents 
    • 374
    What are the 2 types of venous thrombosis 
    • PE 
    375
    What is Virchows triad of clot formation 
    • Hypercoagulability  ## Footnote
    • Blood stasis
    376
    What causes arterial thrombosis 
    • Atherosclerosis - the plaque activates the haemostatic system which triggers thrombosis 
    377
    Give some examples of inherited thrombophilias
    • Factor V leiden 
  • Prothrombin mutation  ## Footnote
  • Protein S deficiency 
  • Antithrombin deficiency 
  • High factor VIII levels 
    • 378
    What is factor 5 leiden 
    • Most common inherited thrombophilia  ## Footnote
    • This means more prothrombin is converted to thrombin which can increase the turnover of fibrinogen to fibrin therefore a clot 
    379
    List some risk factors for acquired thrombosis 
    • Stasis  ## Footnote
    • Prolonged immobility 
    • Stroke 
    • Cardiac failure 
    • Pelvic obstruction 
    • Dehydration 
    • Hyperviscosity 
    • Polycythaemia
    380
    What is TTP
    • Autoimmune condition where AB's are developed against ADAM19 protein which normally regulates Von Willebrand factor 
  • This leads to unregulated thrombus formation and eventually low platelet count
    • 381
    What is the clinical presentation of TTP
    • Fever
    • Renal failure
    • Neurological abnormalities 
  • Thrombocytopenia  ## Footnote
    • 382
    What are the investigations for inherited thrombophilias
    • Direct assays 
  • Protein C and S levels  ## Footnote
  • Mutation analysis of prothrombin gene and Factor V leiden
    • 383
    What are the investigations for thrombocytopenia 
    • Lupus anticoagulant assay  ## Footnote
    • ADAMTD13 assay for TTP
    384
    What is the function of RBCs
    • Transport O2 from lungs to tissue 
    385
    What is the function of platelets
    • Form haemostatic plug to vascular endothelium
    386
    What is the function of neutrophils 
    • Acute inflammatory response to bacterial infections and removal of bacteria via phagocytosis 
    387
    What is the function of basophils 
    • Local inflmmation and allergic reaction - bind to allergen to cause degranulations
    388
    What is the function of eosinophils 
    • Chronic inflammation, allergic reaction and host defence against parasitic infection
    389
    List the agranular white cells 
    • Lymphocytes - B cells, T cells , NK cells  ## Footnote
    • Macrophages 
    390
    What are the 3 types of neutropenia 
    • Mild - 1-1.5
    • Moderate - 1-0.5
    • Severe - <
    391
    What medications cause neutropenia 
    • Abx - cephalosporins, vancomycin and macrolides  ## Footnote
    • Anti malarial 
    • Anti inflammatory - sulfasalzine 
    • Pychotopics - clozapine 
    • Anti arrythmics 
    392
    What is Faconi anaemia 
    • Most common cause of inherited bone marrow failure  ## Footnote
    • Present with - short stature, bone abnormalities, cafe au lait spots, renal/cardiac and GUI malformations 
    • They are high risk of head and neck SCC
    • Can only be cured via haemopeotic stemm cell transplant 
    393
    What is dyskeratosis congenita 
    • A germline mutation resulting in a telomere disease  ## Footnote
    • Many will go on to develop bone marrow failure 
    • Treated with stem cell transplant or steroids
    394
    How do you investigate a neutropenic patient 
    • History - infections, drugs, autoimmunity, B symptoms  ## Footnote
    • Examination 
    • Ix - FBC, blood film, haemantics, viral screen, TFTs, autoimmune screen 
    • CXR
    • USS abdo
    • Bone marrow biopsy 
    395
    What are some causes of congenital neutropenia 
    • Ethnic
    • Severe congential neutropenia/Kostmann syndrome  ## Footnote
    396
    What is severe congenital neutropenia/Kostmann syndrome 
    • ELANE mutation that results in severe neutropenia  ## Footnote
    • Treated with stem cell transplant or granulocyte colony stimulating factor 
    397
    What is cyclical neutropenia 
    • Period form of neutropenia which occurs at regular intervals every 21 days  ## Footnote
    • Frequent fevers and skin/oropharyngeal infections 
    • Respond to granulocyte colony stimulating factor 
    398
    What causes CML
    • Translocation between chromosome 9 and 22 resulting in BCR-ABL gene - mutated tyrosine kinase 
  • 9:22 - Philadelphia chromosome
    • 399
    What are the 3 stages of CML
    • Chronic 
  • Accelerated 
    • 400
    What will an FBC show in CML
    • Leucytosis 
  • Neutrophilia  ## Footnote
  • Thrombocytosis 
  • Blast cells on blood film
    • 401
    What are some benign causes of lymphocytosis 
    • Viral - EBV, CMV, HIV  ## Footnote
    • Smoking
    402
    What are some malignant causes of lymphocytosis 
    • Lymphoproliferative diseases
    • CLL
    403
    What are the investigations for lymphocytosis 
    • Immunophenoyping on peripheral blood  ## Footnote
    • Biopsy of target lesion 
    404
    What are the signs and symptoms of EBV
    • Splenomegaly 
  • Hepatomegaly  ## Footnote
  • Encephalitis 
  • Stiff neck
    • 405
    What is the treatment for EBV
    • Self resolving 
  • Do not get penicillin 
    • 406
    What cells belong to the myeloid lineage 
    • Granulocytes - neutrophils, monocytes, eosinophils and basophils  ## Footnote
    • Platelet
    407
    What cells belong to the lymphoid lineage 
    • T cell 
    408
    What is myeloproliferative neoplasms 
    • Too much of particular type of blood cell is produced
    409
    What is myelodysplastic syndrome 
    • Bone marrow doesnt work properly or creates faulty blood cells
    410
    What does bone marrow failure result in 
    • Anaemia  ## Footnote
    • Neutropenia
    411
    What causes CML
    • Caused by a translocation between chromosome 9 and 22 resulting in the Philadelphia chromosome 
  • This creates the BCR-ABL fusion protein with abnormal tyrosine kinase activity resulting in abnormal proliferation 
    • 412
    List some clinical features of CML
    • B symptoms - weight loss, anorexia, fatigue and night sweats 
  • Splenomegaly  ## Footnote
    • 413
    What are the lab findings of CML
    • Basophilia 
  • Leukocytosis  ## Footnote
  • Lots of granulocytes at diff stages of matiration 
  • Anaemia 
    • 414
    What is the treatment of CML
    • Imantinib - tyrosine kinase inhibitor 
    415
    What is the prognosis of CML
    • 1-2% progresses to AML - poor prognosis 
  • Can present in chronic, accelerates or blast phase
    • 416
    What is the difference between MPN and MDS
    • MPN - increased blood cells 
  • MDS - reduced quality and quantity of blood cells 
    • 417
    Give some examples of MPD's
    • Polycythaemia vera 
  • Essential thrombocythaemia  ## Footnote
  • Myelofibrosis 
    • 418
    What is the common features of MPD's
    • Clonal proliferation 
  • Risk of progression to AML
    • AMLK
    419
    What is essential thrombocythaemia 
    • Increase in platelet count due to megakaryocytes proliferation  ## Footnote
    • May present with thrombosis or haemorrhage, erythromelagia and splenomegaly 
    • Associated with JAK2 mutation 
    420
    What is the management of essential thrombocythaemia 
    • Hydroxycarbamide/hydroxyurea to control platelet count  ## Footnote
    • Asprin to reduce thrombosis risk 
    421
    What is polycythaemia vera 
    • Too many blood cells produced resulting in increase red cell volume 
    422
    List the clinical features of polycythaemia vera
    • Headache 
  • Dyspnoea  ## Footnote
  • Night sweats 
  • Plethora 
  • Bleeding 
  • Splenomegaly 
  • Pruritus
    • 423
    What are the lab features of polycythaemia vera
    • Raised haematocrit and HB 
  • WBC and platelets may also be raised 
    • 424
    What is the treatment of polycythaemia vera 
    • Venesection - remove blood  ## Footnote
    • Hydroxyurea or interferon alpha second line 
    425
    What is primary myelofibrosis 
    • Progressive generalised fibrosis of the bone marrow associated with extramedullary haematopoiesis 
    426
    What are the clinical features of primary myelofibrosis 
    • Anaemia symptoms  ## Footnote
    • B symptoms 
    • Blood film - poikilocytes
    • Initially raised WCC/platelets but as it progresses it becomes pancytopenia 
    • Associated with JAK2 mutation and calreticulin mutation 
    • Dry tape bone marrow biopsy 
    • Treated with JAK2 inhibitors
    427
    What is the management of primary myelofibrosis 
    • Transfusion support  ## Footnote
    428
    What are the clinical features of MDS
    • Common in elderly 
  • Usually slow progression  ## Footnote
    • 429
    What are the lab features of MDS
    • RBCs - macrocytosis, poikilocytosis 
  • Neutrophils - hypogranular, psuedo Pelger  ## Footnote
  • BM - hypercellular, ring sideroblasts and increased blast cells 
    • 430
    What is the management of MDS
    • Transfusion support 
  • Growth factors - granulocyte colony stimulating factor, erythropoietin  ## Footnote
  • Chemotherapy if high risk of AML progression 
  • Stem cell transplant 
    • 431
    What is AML
    • Aggressive clonal neoplastic disorder of the bone marrow 
  • Results in proliferation and arrest of myeloid precursors/blast cells  ## Footnote
  • Affects males more
    • 432
    How is AML diagnosed
    • FBC - neutropenia, anaemia and thrombocytopenia
    • Blood film - blast cells and auer rods 
  • Bone marrow biopsy has to be done to confirm AML
    • 433
    What is the management of AML
    • Supportive care 
  • Chemotherapy  ## Footnote
    • 434
    What is ALL
    • Accumulation of lymphoid blasts in the bone marrow and lymph nodes 
    435
    What are the clinical features of ALL
    • BM failure 
  • Organ infiltration  ## Footnote
  • Rarely any testicular or mediastinal mass
    • 436
    What are the lab features of ALL
    • Lymphoid markers 
  • Philadelphai chromosome in few cases 
    • 437
    What is the treatment for ALL
    • Supportive care - blood prodcuts, antifungals, abx, hickman line etc 
  • Chemo for 2-3 years plus intrathecal methotrexate  ## Footnote
  • CAR-T cellular therapy 
    • 438
    What is CLL
    • Persistent lymphocytosis with increased mature lymphocytes 
    439
    Whar are the clinical features of CLL
    • Lymphadenopathy 
  • Hepatosplenomegaly  ## Footnote
  • B symptoms
  • Asymptomatic and found on a routine FBC
    • 440
    What will a blood film show in CLL
    • Smear cells
    441
    What is the treatment for CLL
    • Active surveillance 
  • Oral chemo  ## Footnote
  • Monoclonal antibodies 
    • 442
    What is lymphoma 
    • Malignant proliferation of lymphocytes that accumulate in lymph nodes 
    443
    What causes lymphoma 
    • Viral - EBV
    • Immune dysregulation - HIV
    • Familial  ## Footnote
    • Gene mutation 
    • Proproliferative or pro-apoptotic
    444
    What are the general clinical features of lymhoma
    • B symptoms (fever, night sweats or weight loss)
    • Malaise
    • Painless lymphadenopathy
    • Hepatosplenomegaly
    • Mass
    • Mass effects → hydronephrosis
    445
    How is lymphoma diagnosed
    • CT/MRI 
  • Lymph node bipsy - excise whole node or fine needle aspirate  ## Footnote
  • Bone marrow biopsy 
    • 446
    How is lymphoma staged "Ann Arbour
    "">
  • 1 → 1 lymph node group
  • 2 > ## Footnote "">A for no B symptoms and B for b symptoms present

    " What is the prognosis of lymphoma
    • Hodgkin → agressive but curable
    • High grade NHL → often curable
    • Low grade NHL → indolent and treatable but not curable 
    What is the treatment of lymphoma
    • chemotherapy
    • Immunotherapy
    • Small molecule inhibitor
    • Radiotherapy
    • Stem cell transplant
    • Supportive care
      • Surgery is not a treatment of choice
    What is hodgkin lymphoma
    • High grade lymphoma associated with Reed sterberg cells 
    • EBv detected in 50% of cases
    List the features of Hodgkin lymphoma
    • Any age
    • Biomodal peak
    • Male - female 2:1
    • Lymphadenopathy - painless
    • Splenomegaly
    • B symptoms & pruritis
    What is the treatment for hodgkin lymohoma
    • chemotherapy
      • ABVD = Adriamycin® (doxorubicin), bleomycin, vinblastine and dacarbazine
    • Monoclonal antibodies
    • Checkpoint inhibitors
    • Radiotherapy
    • Stem cell transplant
    What are the types on non Hodgkin lymphoma 
    • Low grade - follicular lymphoma 
    • Mantle cell lymphoma 
    • High grade - Diffuse large B cell lymphoma and burkitts lymphoma
    What is the treatment for non hodgkin lymphoma 
    • Low grade - active surveillance and treat if symptomatic 
    • High grade - chemo, monoclonal antibodies, radiotherapy, stem cell transplant
    What are T cell lymphomas
    • Rare 
    • Present with peripheral lymphadenopathy
    • Adult T cell Leukaemia associated with HTVL-1 infection
    What is myeloma 
    • B cell lymphoid malignancy characterised by monoclonal expansion and accumulation of abnormal plasma cells in the bone marrow
    What is the clinical presentation of myeloma
    • Hypercalacaemia 
    • Renal impairment 
    • Anaemia 
    • Bone pain
    • Hyperviscosity 
    • Amyloidosis 
    • Infection 
    • Bleeding/bruising 
    What are the investigations for myeloma
    • FBC
    • U&E
    • LFTs
    • Immunoglobulins
    • Serum protein electrophoresis - M band 
    • Beta-2 micro globulin
    • Creatinine clearance
    • Urinary protein electrophoresis and immunofixation
    • Quantification of BEnce Jones Proteins
    How does myeloma bone disease present 
    • Lytic lesions
    • Pathological fractures
    • Spinal cord compression 
    How can you diagnose myeloma histologically 
    • Bone marrow aspirate 
    • Bone marrow trephine 
    • Cytogenetics 
    • Flow cytometry 
    List some complications of myeloma
    • anaemia / bone marrow failure
    • Myeloma bone disease
    • Spinal chord compression
    • Renal failure
    • Hypercalcaemia
    • Hyperviscosity syndrome
    • Infections
    • Amylodoisis
    • Bleeding
    Outline renal failure in myeloma
    • immunoglobulins deposit → acute tubular necrosis
    • Iatrogenic → NSAIDS
    • Pyelonephritis
    • Hypercalcaemia
    • Hyperuricaemia
    • Amyloid
    What is the treatment for myeloma
    • Active surveillance
    • Steroids
    • Targeted therapies
    • Chemotherapy
    • Radiotherapy
    • Supportive care
    • Phphoplasty
    How does bisphosphonates treat myeloma
    • Inhibits osteoclasts activity by preventing differentiation 
    • Induces apoptosis in myeloma in vitro
    What are the physiological changes in pregnancy
    • physiological anaemia
    • Neutrophils
    • Mild thrombocytopenia
    • Increased procoagulant factors
    • Diminished Fibrinolysis
    How does haemoglobin change in pregnancy
    • Plasma volume increases 
    • Circulating RBC increases 
    • Physiological fall in Hb - dilutional anaemia 
    What are the Hb ranges in pregnancy 
    • Hb <110g/l in first trimester
    • <105 g/L in second and third trimester
    • <100 g/l in post party period
    What are the 3 types of anaemia in pregnancy 
    • Dilutional 
    • Iron deficiency 
    • Folic acid deficiency 
    What are the impacts of iron deficiency anaemia in pregnancy
    • Impaired psychomotor and mental development 
    • Preterm delivery 
    • Low birth weight 
    How is iron deficiency in pregnancy diagnosed 
    • Ferritin <15 ug/l + transferrin saturation <15%
    • Microcytosis and hypochromia
    When would you start oral iron in iron deficiency anaemia pregnancy 
    • Ferritin less than 30
    What is the management of iron deficiency anaemia in pregnancy 
    • Ferrous sulphate 200mg 
    • Take on empty stomach 1hr before meal 
    • Take with source of vitamin c 
    How can folate deficiency be managed
    • 400ug daily preconception 
    • 5mg daiky if previosu neural tube defects, DM etc 
    Outline neutrophilia in pregnancy "
    • neutrophil count begins to increase in the  second  month of pregnancy 
    • WCC falls to baseline by sixth day postpartum
    "
    • 447
    Outline thrombocytopenia in pregnancy
    • Falls during pregnancy 
  • Happens due to dilutional effects secondary to increase plasma volume  ## Footnote
    • 448
    What is gestational thrombocytopenia 
    • Happens mid to late tirmester  ## Footnote 80
    • Resolves after birth 
    • Diagnosed via exclusion 
    449
    Outline ITP in pregnancy 
    • Autantibodies to platelets 
    450
    What is the inidications for management of ITP in pregnancy 1-2 trimester
    • Symptomatic bleeding
    • Platelet count <
    30
  • Planned procedure
    • 34-36 weeks
    • Treatment is aiming to reduce risk of maternal haemorrhage at delivery
    • Platelets count 50-80
    451
    What are the preauations during delivery in ITP "for mother
    • Epidural is contraindicated with platelet count <
    80
  • C section aim for platelet > ## Footnote "">For baby:
    • fetal scalp electrodes
    • Ventouse delivery
    • Rotational Forceps
    " What are the haemostatic changes in pregnancy 
    • inc conc of clottimg factors 
    • Decreased conc of some anticoags 
    • Diminished fibrinolytic activity

    How do coagulation inhibitors change in pregnancy
    • Decreased protein S
    • Unchanged / Slight ? Decrease → Protein C
    • Antithorbin → unchanged or slight decrease
    What is the VTE prophylaxis in pregnancy 
    • Moderate risk - 6 weeks post natal prophylactic LMWH 
    • High risk - Prophylactic LMWH antenatal + 6 weeks post partum 
    • Very high risk - antenatal therapeutic LMWH and 6 weeks post partum plus specialist management 
    Why is LMWH used in pregnancy
    • Does not cross placenta
    • But you will need to stop it during delivery or epidural anaesthesia
    • Given as Dalteparin 90 units 
    • LMWH and warfarin are safe in lactating mothers
    List some haem complications in pregnancy 
    • antiphopholipid syndrome
    • Thrombocytopenia disorders
    • HELLP / PET / DIC
    • Bleeding disorders
    • Thrombotic disorders
    • Sickle Cell disease
    • Myeloproliferative Disorders
    How does Hb change in children 
    • 3rd trimester - rising Hb and MCV and HbF 
    • Birth - HbA production and HbF switched off 
    • 8-12 weeks - Hb increases and start making own RBC
    Outline neonatal anaemia 
    • 28 weeks - marked macrocytosis and plentoful nucleated RBC
    List some causes of neo natal anaemia 
    • Haemolysis - most common 
    • Blood loss 
    • Decreased production 
    What are the lab investigations for neonatal anaemia 
    • FBC, retic, DAT blood film, group and save 
    • Maternal antibodie screen 
    • Kleihaurs test 
    • Cranial ultrasound 
    • Parvovirus PCR 
    Outline the pathophysiology of haemolytic disease of the newborn 
    • Foreign blood enters womans circulation 
    • The foreign antigen is recognised 
    • Mother makee antibody agaisnt the RBC antigen 
    • AB crosses the placenta and if specficic to babys RBC then AB bind and haemolysis occurs 
    How is HDN prevented 
    • Early identification via maternal antibody screen 
    How is HDN managed 
    • Intrauterine transfusion 
    • Time the delivery 
    Give some examples of RBC membrane disorders
    • Hereditary spherocytosis 
    • Hereditary elliptocytosis 
    • Hereditary pyropoikilocytosis
    What is G6PD deficiency
    • X linked deficiency 
    • G6PD protects cells from oxidative stress
    • Can be cuased by fava beans
    What are the clinical features of G6PD deficiency
    • Persistent neonatal jaundice 
    • Anaemia 
    • Dark/tea coloured urine 
    • Splenomegaly 
    • Bite cells, blister cells and irregularly contracted cells 
    What is the treatment for G6PD deficiency
    • Removal of trigger - treating illness or discontinuing drug 
    • Severe anaemia may require blood transfusion
    What causes alloimmune thrombocytopenia 
    • Paternal platelet antigen on fetal platelet 
    • Fetal platelet enter maternal circulation
    • Maternal platelets do not recognise the antigen therefore foetal ones are foreign 
    • Mother makes antibodies against antigen - alloimmunisation 
    • IgG antibody crosses the placenta 
    • This destrys fetal platelets resulting in fetal thrombocytopenia +/- bleeding
    What is haemorrhagic disease of the newborn
    • Vitamin K deficiency bleeding 
    • Treated with IM/oral Vit K, FFP if life threatening or blood trasnfusion if massive bleed 
    • Prevention: routine administeration of Vit K 1mg IM within 1 hour of birth
    What contributes to anaemia of chronic disease
    • Blood loss
    • Poor nutrition 
    • Immune 
    • Red cell fragmentation 
    • Polycythaemia
    What are the characteristics of ACD
    • Disordered iron metabolism 
    • Reduced red cell lifespan 
    • Reduced bone marrow response to erythropoietin
    What is hepcidin
    • Iron regulatory protein to stop the absorption of uron from the gut and release from macrophages 
    • It will inhibit ferroportin which is a trasnmembrane protein responsible for transporting iron from inside the cell to outside
    How is ACD diagnosed
    • Normocytic normochromic anaemia 
    • Iron studies - low serum iron, low transferrin saturation, reduced TIBC, increased ferritin
    • Increased macrophage iron store - iron stain on BM sample 
    What are the differences between ACD and iron deficiency
    • MCV - normal vs low
    • Serum iron - both reduced 
    • Transferrin saturation - both reduced 
    • TIBC - reduced vs increased 
    • Ferritin - increased vs reduced 
    • BM iron stores = present vs absent 
    • Hepcidin - increased vs decreased 
    • CRP/ESR - increased vs decreased
    What is TIBC
    • Measurement of the capcity of transferrin to bind to iron 
    • In IDA - more trasferrin produced to transport more iron to tissue therefore TIBC high 
    • ACD - less trasnferrin produced to reduce availability of iron for pathogens therefore TIBC low
    What results suggest haemolysis  "
    • Unconjugated hyperbilirubinaemia
    • RBC fragments
    • Raised LDH
    • Increased reticulocytes
    " What codnitions may have ACD
    • Infection → TB
    • Cancer
    • Autoimmune & connective tissue → Vasculitis. SLE
    • CKD
    • Congestive heart disease
    What is the treatment of ACD
    • iron suplementation
    • Trial of EPO
    • Blood transfusion
    What is APML
    • Acute promyelotic leukaemia
    • Rare type of AML caused by translocation between chromosome 15 and 17 resulting in disruption of retinoid acid receptor
    • Marked increase in promyelocytes
    • Can present with DIC
    What bacteria are people with sickle cell suscpetibel to 
    • Streptococcus pneumonia 
    • Psuedomonas aeruginosa 
    • Haemophilus influenzae

    IOD (2 decks)

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