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Haematology Flashcards

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1
Q

How can myelodysplastic syndrome be distinguished from AML?

A

Percentage of blast cells in bone marrow
<20% myelodysplastic syndrome
>20% AML

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2
Q

What is an auer rod and which condition do you see them in?

A

Granular material that forms elongated needles in cytoplasm of myeloid leukaemic blasts
Myeloperoxidase aggregate

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3
Q

What are smudge/smear cells and what condition do you see them in?

A

Remnants of cells that lack identifiable cytoplasmic membrane or nuclear structure
Associated with fragile lymphocytes in conditions like CLL

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4
Q

Which leukaemia is response to tretinoin therapy?

A

Acute promyelocytic leukaemia

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5
Q

What is myelofibrosis?

A

Proliferation of abnormal clone of haematopoietic stem cells in bone marrow and other sites leads to fibrosis

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6
Q

What are signs and symptoms of myelofibrosis?

A 
Splenomegaly
Bone pain
Bruising and easy bleeding
Cachexia 
Hepatomagaly 
Fatigue 
Gout and high uric acid levels 
Increased susceptibility to infection
Pallor and SOB - anaemia
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7
Q

Why does malignant hypertension lead to haemolytic anaemia?

A

Mechanical - red blood cells rupture as they become damaged when forced at high pressures through microvasculature

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8
Q

What makes autoimmune haemolytic anaemias warm or cold?

A

Description of what temperatures the antibodies against the RBCs best work

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9
Q

What blood test abnormalities would you expect to find in DIC?

A

Increased PT and APTT
Anaemia
Thrombocytopenia
Increased fibrin degradation products

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10
Q

What are some causes of DIC?

A

Leukaemias
Serious infections - meningococcus, E. coli
Obstetric emergencies

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11
Q

What are signs and symptoms of an acute haemolytic transfusion reaction?

A

> 2 degrees rise in temperature
Abdominal pain
Hypotension

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12
Q

What features are typical of Von Willibrands disease?

A 
Lifelong tendency to easy bruising
Frequent epistaxis
Menorrhagia 
Purpura
Post op bleeding
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13
Q

Which clotting result would be deranged in Von willebrands disease?

A

Prolonged activated partial thromboplastin time

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14
Q

What can be precipitants of haemolysis in G6PD deficiency?

A

Consumption of fava beans
Antimalarials
Nitrofurantoin

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15
Q

What is marrow involvement in Hodgkin’s disease best treated with?

A

Chemotherapy

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16
Q

What can cause a raised ESR?

A

RA
SLE
Polymyalgia rheumatica
Myeloma

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17
Q

What happens to ESR with age?

A

Increases

ESR normally 1/2 age

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18
Q

What happens to ESR in polycythemia?

A

Low

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19
Q

A 6 month old boy was noticed to be pale but otherwise well and gaining weight. The liver was 3cm below costal margin and 2cm spleen palpable. Haemoglobin 80, reticulocyte count 4%, WCC normal. What is the likely diagnosis? Why?

A

Beta thalasseamia major

Anaemia, extramedullary haematopoiesis, hepatosplenomegaly and raised reticulocytes

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20
Q

What does chloramphenicol toxicity do to bone marrow?

A

Complete suppression

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21
Q

What clinical features does G6PD deficiency cause?

A

Episodes of haemolysis - jaundice, abdominal pain, dark urine

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22
Q

What is the commonest presenting symptom of myeloma?

A

Bone pain

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23
Q

What causes polyuria in myeloma?

A

Hypercalcaemia due to bone destruction

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24
Q

What are the main antigens that give rise to graft rejection?

A

ABO blood group
Human leukocyte antigen (HLA)
Minor histocompatibility antigens

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25
What are some causes of hyperacute organ rejection?
Blood group ABO incompatibility | Pre existing HLA antibodies
26
What are the 4 most important HLA types in organ rejection?
HLA A HLA B HLA C HLA DR
27
If a patient is HLA mismatched with their donor for organ transplant, what will happen?
T lymphocytes will recognise antigens bound to HLA molecules and become activated Clonal expansion occurs with a response directed at that antigen
28
What are the different types of organ rejection?
Hyperacute: immediately through pre formed antigens Acute: during first 6 months, usually T cell mediated, tissue infiltrates and vascular lesions, mononuclear cell infiltrates Chronic: after 6 months, vascular changes predominate, myointimal proliferation leading to organ ischaemia, loss of acinar cells in pancreas, rapidly progressive CAD in cardiac transplants
29
Which transplants are at the highest risk of hyperacute organ rejection?
Renal
30
Give some examples of immune mediated blood transfusion reactions
``` Pyrexia Alloimmunisation Thrombocytopenia Transfusion associated lung injury Graft versus host disease Urticaria Acute or delayed haemolysis ABO incompatibility Rhesus incompatibility ```
31
Give some examples of non immune mediated blood transfusion reactions
Hypocalcaemia CCF Infections Hyperkalaemia
32
What is graft versus host disease?
Lymphocyte proliferation causing organ failure
33
What is transfusion associated lung injury?
Neutrophil mediated allergic pulmonary oedema
34
What are some oral signs of AIDS?
Oral candidiasis Hairy leukoplakia Oral gingival regression Kaposis sarcoma
35
What is wrong with the haemoglobin of patients with sickle cell anaemia? What is different with sickle cell trait?
Substitution of valine for glutamic acid at position 6 of the beta chain Abnormal haemoglobin depolymerises at PO2 of 5-6 kPa which is found in normal venous blood so they are continuously sickling Heterozygotes contain normal and abnormal Hb and only sickle at extremely low PO2 values of 2.5-4kPa
36
What is important in the peri operative management of sickle cell disease patients?
``` Keep well oxygenated Keep warm Keep them hydrated Provide adequate analgesia with PCA Avoid acidosis (venous stasis) ```
37
What can precipitate sickling in sickle cell disease?
``` Hypoxaemia Hypothermia Dehydration Infection Exertion Stress ```
38
What are clinical features of multiple myeloma?
``` Bone disease: pain, osteoporosis, pathological fractures, osteolytic lesions Lethargy Infection Hypercalcaemia Renal failure Amyloidosis Carpal tunnel syndrome Hyperviscosity ```
39
What investigations should be done for multiple myeloma?
Monoclonal proteins: IgG or IgA in serum and urine (bence jones proteins) Increased plasma cells in bone marrow Bone lesions on skeletal survey
40
What are the criteria for diagnosis of multiple myeloma?
Major: plasmacytoma, 30% plasma cells in bone marrow sample, elevated levels of M protein in blood or urine Minor: 10-30% plasma cells, minor elevations in M protein, osteolytic lesions, low levels of antibodies in blood Diagnosis requires 1 major and 1 minor or 3 minor in someone with signs and symptoms
41
Why does hypercalcaemia occur in multiple myeloma?
Increased osteoclastic bone resorption caused by local cytokines released by myeloma cells Impaired renal function Increased renal tubular calcium reabsorption and elevated PTHrP levels
42
What features might be seen on a blood film in a hyposplenic patient?
``` Target cells Howell Jolly bodies Pappenheimer bodies Siderotic granules Acathocytes ```
43
What features might be seen on a blood film of a patient with iron deficiency anaemia?
Target cells | Pencil poikilocytes
44
What feature is seen on a blood film of a patient with myelofibrosis?
Tear drop poikilocytes
45
What feature is seen on blood film of a patient with intravascular haemolysis?
Schistocytes
46
What is seen on a blood film of a patient with megaloblastic anaemia?
Hypersegmented neutrophils
47
What is the most common inherited bleeding disorder?
Von Willebrands disease
48
What are common features of Von willebrands disease?
Epistaxis Menorrhagia Bleeding after tooth extraction
49
What are the different types of Von willebrands disease?
Type 1: partial reduction in vWF Type 2: abnormal form Type 3: total lack of (autosomal recessive)
50
How is Von willebrands investigated?
Prolonged bleeding time APTT may be prolonged Factor VIII levels moderately reduced Defective platelet aggregation with ristocetin
51
How is Von willebrands managed?
Tranexamic acid for mild bleeding Desmopressin: raises levels of vWF by inducing release from Weibel-Palade bodies in endothelial cells Factor VIII concentrate
52
What are the 4 different crises of sickle cell anaemia?
Thrombotic (painful) Sequestration Aplastic Haemolytic
53
What is a thrombotic crisis in sickle cell anaemia?
Painful, vaso occlusive Precipitated by infection, dehydration, deoxygenation Infarction organs including bones
54
What is sequestration crisis in sickle cell anaemia?
Sickling within organs such as spleen or lungs causes pooling of blood with worsening of anaemia Acute chest syndrome: dyspnoea, chest pain, pulmonary infiltrates, low PO2
55
What is the most common cause of death after childhood in sickle cell anaemia?
Acute chest syndrome
56
What is aplastic crisis in sickle cell anaemia?
Infection with parvovirus | Sudden fall in haemoglobin
57
What is a haemolytic crisis in sickle cell anaemia?
Rare | Fall in haemoglobin due to increased haemolysis
58
What are the histological classes of Hodgkin's lymphoma? Which has the best and worst prognosis?
Nodular sclerosing - good prognosis Mixed cellularity - good prognosis Lymphocyte predominant - best prognosis Lymphocyte depleted - worst prognosis
59
What are B symptoms of Hodgkin's lymphoma?
Weight loss >10% in last 6 months Fever >38 Night sweats
60
What factors are associated with poor prognosis of Hodgkin's lymphoma?
``` B symptoms Age >45 Stage IV disease Haemoglobin <10.5 Lymphocyte count <600 Male Albumin <40 White cell count >15000 ```
61
What is Burkitts lymphoma?
High grade B cell neoplasm Endemic (Africa): maxilla or mandible Sporadic: abdominal (ileo-caecal), commonly with HIV
62
What gene translocation is associated with Burkitts lymphoma?
C myc t(8:14)
63
Which virus is strongly implicated in the development of the African form of Burkitts lymphoma?
Epstein Barr virus
64
What are microscopy findings in Burkitts lymphoma?
Starry sky appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells
65
How is Burkitts lymphoma managed? What problem can this cause?
Chemotherapy | Rapid response which may cause tumour lysis syndrome
66
What is given before chemo in Burkitts lymphoma to reduce the risk of tumour lysis syndrome occur if?
Rasburicase
67
What are complications of tumour lysis syndrome?
``` Hyperkalaemia Hyperphosphataemia Hypocalcaemia Hyperuricaemia Acute renal failure ```
68
What is the Philadelphia chromosome?
t(9:22)(q34;q11) Associated with CML Results in tyrosine kinase activity in excess of normal
69
A 68 year old male presents to GP with enlarged nodes in neck, axillae and groin. Blood tests show Hb 105, WBC 19. A blood film shows 60% white cells are small mature lymphocytes. What is the most likely diagnosis?
Chronic lymphocytic leukaemia
70
What are features of CLL?
``` Often none Constitutional: anorexia, weight loss Bleeding Infections Lymphadenopathy ```
71
What are complications of CLL?
Hypogammaglobulinaemia leading to recurrent infections Warm autoimmune haemolytic anaemia Transformation to high grade lymphoma (Richters transformation)
72
What investigations are done for CLL?
Blood film: smudge cells/smear cells | Immunophenotyping
73
What is the Ann Arbor staging for non hodgkins lymphoma?
Stage 1: involvement of single lymph node region Stage 2: involvement of 2 or more regions on same side of diaphragm Stage 3: involvement of lymph node regions on both sides of the diaphragm Stage 4: multifocal involvement of 1 or more extralymphatic sites +/- associated lymph nodes
74
Which vaccines are live attenuated and therefore shouldn't be given to patients who are immunosuppressed?
``` BCG MMR Oral polio Yellow fever Oral typhoid ```
75
By how much does 1 unit of blood increase Hb concentration?
10g/L
76
What are the most important symptoms/complications of myeloma?
``` CRAB Calcium raised Renal failure Anaemia Bone pain ```
77
What is the difference between warm and cold agglutinins?
Autoantibodies react with red cells at temps above 37 in warm and below 37 in cold Both can be lymphoproliferative disorders Warm primarily in spleen, IgG Cold: infections (mycoplasma/EBV)
78
What is the diagnosis in a 7 year old boy presenting with haemarthrosis of his left knee. He has had repeated episodes of this since his early years. His APTT is prolonged but bleeding time is normal?
Haemophilia A
79
How are minor and major bleeds associated with haemophilia treated?
Minor: desmopressin which raises factor VIII levels Major: factor VIII concentrate to raise levels above 50%
80
What is the management for an alcoholic patient presenting with ruptured oesophageal varices who has received 6 units of blood but has a prolonged prothrombin time?
Fresh frozen plasma to replace clotting factors
81
A day old baby who's mother takes anticonvulsants is admitted with unexplained bruising. Clotting profile shows PT and APTT are prolonged. What is the likely diagnosis and what is the management?
Haemorrhagic disease of the newborn | Give Vit K to correct absence of Vit K dependent clotting factors
82
What is coombs test?
Direct and indirect Direct used for autoimmune haemolytic anaemia Indirect used for prenatal testing of pregnant woman and prior to transfusion
83
What are poor prognostic features of acute myeloid features?
>60 years >20% blasts after first course of chemo Cytogenetics: deletions of chromosome 5 or 7
84
What is Waldenstroms macroglobulinaemia?
Lymphoplasmacytic lymphoma Affects B cells which produce excess immunoglobulin - monoclonal IgM paraproteinaemia Bone marrow infiltration Splenomegaly Lymphadenopathy Hyperviscosity of blood so risk of strokes/clots
85
Why does alcohol cause a large MCV?
Alcohol has effect on alkaline phosphatase on RBC membrane producing relative swelling of the cell
86
What condition is suggested by a prolonged APTT and thrombocytopenia with a background of recurrent DVT?
Antiphospholipid syndrome
87
Name some clotting disorders which make you pro thrombotic (thrombophilias)
``` Factor V Leiden Prothrombin mutation Antithrombin III deficiency Protein C deficiency Protein S deficiency Factor XIII mutation Familial dysfibrinogenaemia Antiphospholipid syndrome ```
88
What is hereditary spherocytosis?
Autosomal dominant abnormality of erythrocyte membrane proteins which interfere with cells ability to travel through capillaries More prone to rupture, cells taken to spleen Haemolytic anaemia Howell Jolly bodies on blood smear
89
What is Virchows triad which describes factors that contribute to thrombosis?
Venous stasis Endothelial damage Blood hypercoagulability: hyperviscosity, deficiency of antithrombin III, nephrotic syndrome, disseminated malignancy, pregnancy, smoking
90
What should be done to manage a case of DIC?
Clotting studies and platelet count Advice from haematologist Up to 4 units of FFP and 10 units of cryoprecipitate whilst awaiting results
91
What is waldenstrons macroglobulinaemia?
Lymphoplasmocytic lymphoma High levels of IgM Preceded by IgM monoclonal gammopathy of undetermined significance Weakness, fatigue, weight loss, chronic oozing of blood from the nose and gums
92
What is porphyria cutanea tarda?
Presents with skin manifestations later in life Low levels of enzyme responsible for 5th step in heme production Onycholysis and blistering of sun exposed skin
93
Which cytokine is involved in the reaction to a Mantoux test?
Interferon gamma secreted by t helper 1 cells which stimulate macrophage activity
94
In which condition are smudge cells seen on blood film?
Chronic lymphocytic leukaemia
95
What is the most common childhood malignancy?
Acute lymphoblastic leukaemia
96
Which cell lineage is most commonly affected in ALL?
B cell lineage
97
What are some causes of AML/ALL?
Ionising radiation Chemicals Viruses Chromosomal abnormalities
98
What are clinical features of ALL?
Critically ill due to bone marrow failure: Anaemia, Infection, Haemorrhage Signs of leucostasis Bone/joint pain Widespread lymphadenopathy Mild to moderate hepatosplenomegaly + orchidomegaly CNS involvement – meningism, facial nerve palsy
99
What investigations should be used for ALL and what might the results show?
``` FBC – Hb, neutrophils and platelets low Total WCC high with blast cells on film Blood film – blast cells Bone marrow aspirate - >20% blast cells CXT/CT – mediastinal/abdominal lymphadenopathy LP – CNS involvement ```
100
What is the management of ALL?
Resuscitation if septic / shock / haemorrhage Supportive treatment Chemotherapy: Aim to eliminate leukaemic cells and achieve complete haematological remission 4 continuous phases: Remission induction, Consolidation therapy, CNS prophylaxis, Maintenance therapy Consideration of allogenic stem cell transplantation
101
What are poor prognostic factors in ALL?
Patient age High leucocyte count Cytogenetics Long time to complete remission (>4 weeks)
102
What is the most common leukaemia in adults?
AML
103
What are associated conditions with AML?
Myelodysplasia, prior chemotherapy, radiation, chromosomal abnormalities (Down syndrome)
104
What are clinical features of AML?
Present critically ill due to bone marrow failure Anaemia: Weakness, lethargy, breathlessness, palpitations Infection: Chest, mouth, perianal, skin Haemorrhage: Purpura, epistaxis, bleeding gums Gum hypertropy / skin infiltration Hepatosplenomegaly Leucostasis: Hypoxia, confusion, CVA
105
What investigations should be done for AML?
Bloods: FBC – leukocytosis, anaemia, thrombocytopaenia Blood film – presence of blast cells Bone marrow aspirate - >20% blast cells Differentiate from ALL with immunophenotyping or cytoplasmic myeloperoxidase (+ve in AML)
106
What is the management of AML?
Resuscitation – treat for neutropenic sepsis, haemorrhage Fluids/allopurinol for tumour lysis syndrome Chemotherapy: Aim to achieve complete haematological remission (<5% blast cells) + normalisation of peripheral blood count 2 phases: Remission induction – 2 cycles of combination chemo, Consolidation therapy – 2 cycles – reduce risk of relapse
107
What are poor prognostic factors for AML?
Advancing age >60 years Specific cytogenetic abnormalities – ‘poor risk’ groups Failure to achieve complete remission with 1st cycle
108
What is chronic lymphocytic leukaemia?
Progressive accumulation of mature appearing B lymphocytes in theperipheral blood, bone marrow, lymph nodes, spleen and liver
109
What are clinical features of chronic lymphocytic leukaemia?
Asymptomatic – incidental lymphocytosis on routine FBC Advance disease associated with: Weight loss, fever, malaise, Lymphadenopathy, hepatosplenomegaly Bone marrow failure due to infiltration: anaemia, neutropenia, thrombocytopenia Recurrent infections due to reduced antibody production: Hypogammaglobulinaemia
110
What investigations should be done for CLL? What might they show?
FBC – lymphocytosis > 5.0, Autoimmune haemolysis, Thrombocytopenia, anaemia, neutropenia Blood film: Lymphocytosis, Presence of ‘smear’ / ‘smudge’ cells – damage to cells during preparation Immunophenotyping BM - >30% mature lymphocytes Immunoglobulins – hypogammaglobulinaemia
111
What is the management of CLL?
Choice between observation, palliation or aim for complete remission Management based on age, performance status, co-morbidities, symptoms present and prognostic factors Palliative approach treating symptomatology with minimal toxicity OR prolong disease-free survival Palliative - alkylating agents
112
What is the prognosis of CLL?
Incurable disease | Early stage, asymptomatic patients – die of unrelated causes Advance stage - refractory disease and bone marrow failure
113
What are poor prognostic factors for CLL?
Male Initial lymphocytosis >50 x 109/L Lymphocyte levels doubling in <12 months Specific cytogenetic abnormalities/ mutations Poor response to therapy
114
What is CML?
Characterised by large granulocytes and presence of the Philadelphia chromosome
115
What are clinical features of CML?
``` Accelerated phase characterised by blood count and organomegaly becoming refractory to therapy 30% asymptomatic at diagnosis Lethargy, weight loss, sweats Splenomegaly, bruising/bleeding Occasional signs of leucostasis ```
116
What investigations should be done for CML and what might they show?
FBC: Raised WCC (often 100-300 x 109/L) – predominantly neutrophils, Anaemia, Platelets often unaffected or raised Raised urate BM: Hypercellularity – granulocyte hyperplasia, Chronic phase <10% blasts / accelerated >10% / crisis >20% Cytogenetic analysis: Presence of Philadelphia chromosome
117
What is the management of CML?
Commence allopruinol Consideration of leucapheresis in leucostasis Tyrosine kinase inhibitor (Imatinib) – 1st line – turns off signalling cascade
118
What are the myeloproliferative disorders?
Polycythemia vera – excessive proliferation of the erythrocyte, myeloid and megakaryocyte lineages Essential thrombocythaemia – persistent thrombocytosis not due to any other cause Primary myelofibrosis – neoplasm of early haematopoietic stem cell leading to marked hyperplasia of abnormal megakaryocytes and monocytes stimulating marrow fibrosis
119
What are important features of management of polycythemia vera?
Venesection | Manage thrombosis risk
120
What are features of myeloma?
Usual presentation with bone pain (back) or pathological fracture Weakness, fatigue, thirst, polyuria, oedema due to renal impairment Acute hypercalcaemia HyperCalcaemia, Renal impairment, Anaemia, Bone problems (CRAB)
121
What investigations should be done for myeloma and what might they show?
FBC – anaemia / U+Es – renal impairment Serum protein electrophoresis – detect paraproteins X-ray site of bone pain – pathological fracture / lytic lesions BM aspirate – plasma cell infiltration
122
What is the management of myeloma?
Not curable Transient responses to chemotherapy Pain control Management of complications: Renal impairment, Hypercalcaemia, RTx for bone disease, Anaemia, Cord compression
123
What are causes of DIC?
Sepsis/trauma/organ necrosis/malignancy/liver failure/transfusionin compatibility
124
What factors can be used in a scoring system to help with diagnosis of DIC?
Plt count D-dimer Prolonged PT Fibrinogen level
125
What is the management of DIC?
Treat precipitating cause Supportive therapy Replacement therapy of platelets, cryoprecipitate, FFP
126
What is thrombotic thrombocytopenic purpura?
Increased platelet aggregation and occlusion of arterioles and capillaries of the microcirculation due to antibodies that activate the immune system to inhibit the ADAMTS13 enzyme
127
What are clinical features of TTP?
``` Severe thrombocytopenia Neurological involvement Renal impairment Fever Haemolysis (micro-angiopathic haemolytic anaemia) ```
128
What is the management of TTP?
``` Haematological emergency May require ITU level of care Plasmapheresis FFP for fluid replacement Platelet transfusions can worsen condition therefore only give in major bleed Steroids ```
129
In which condition are Auer rods seen?
AML
130
What are Howell jolly bodies? When are they seen?
``` Blue black inclusions of red blood cells which represent residual nuclear fragments Seen in asplenic patients Haemolytic anaemia Leukaemia Thalassaemia ```
131
What are pappenheimer bodies, when are they seen?
Violet staining granules in periphery of RBCs containing siderblastic bodies Seen in sideroblastic anaemia, lead poisoning, thalassaemia, asplenic patients
132
What is the pathophysiology behind beta thalassaemia major?
Not enough b chain produced Alpha chains precipitate in erythroblasts and mature red cells Ineffective erythropoiesis and haemolysis Anaemia occurs at 3-6 months when switch from g to b chain production takes place Marrow hyperplasia to compensate leads to thalassaemic facies: hair on end appearance of skull X-ray and thinning of cortex with increased risk of fractures
133
Which atypical pneumonia can be associated with cold agglutinins and therefore Raynauds and autoimmune haemolytic anaemia?
Mycoplasma
134
Which crises can occur with sickle cell anaemia?
Haemolytic crisis Splenic sequestration crisis Aplastic crisis
135
To which bugs are patients with sickle cell susceptible?
Encapsulated bacteria due to hyposplenism: pneumococci, meningococci
136
How long after a DVT are patients recommended not to fly for?
2 weeks
137
What is the triad of symptoms in yellow nail syndrome?
Lymphoedema Pleural effusions Yellow discolouration of nails
138
What are indications for red cell transfusion?
Acute blood loss: especially if >1.5 L in adult Symptomatic anaemia with no easily treatable cause Long term transfusion dependent anaemia Radiotherapy patient Hb <100 Chemotherapy patient Hb <90
139
What are potential complications from a blood transfusion?
``` Acute haemolytic reaction Anaphylaxis Transfusion associated lung injury Non haemolytic reaction Allergic reaction Fluid overload ```
140
What is the name of the haemovigillance scheme that monitors adverse events in blood transfusion?
SHOT
141
Which drugs should be co prescribed alongside a blood transfusion?
Hydrocortisone Chlorpheniramine Furosemide
142
In which patients can giving vitamin K cause haemolysis?
G6PD deficiency
143
What is octaplex used for?
Reversal of warfarin therapy or vit K deficiency
144
What is octaplex?
Prothrombin complex concentrate
145
After giving octaplex, when should you re check INR?
30 mins
146
What causes transfusion related lung injury?
Donor Ab (esp HLA or neut) react with recipient WBCs leads to lung infiltrate Occurs 1-6 hours post transfusion Associated with FFP
147
What are indications for FFP?
Coagulation factor deficiency where specific concentrates not available Multiple clotting factor deficiency: liver disease, DIC, massive transfusion
148
What is the greatest cause of transfusion death in the U.K.?
Transfusion associated circulatory overload
149
What are causes of massive splenomegaly?
``` Myelofibrosis CML Visceral leishmaniasis Malaria Gauchers syndrome ```
150
With which condition is the Philadelphia chromosome associated?
CML
151
How does CML present?
``` Middle age Anaemia Weight loss Abdo discomfort Splenomegaly Spectrum of myeloid cells in peripheral blood Decreased leukocyte alkaline phosphatase ```
152
What is the management of CML?
Imatinib: tyrosine kinase inhibitor Hydroxyurea Interferon alpha Allogenic bone marrow transplant
153
What is the maximum time for transfusion of red cells?
3.5 hours
154
When would you prescribe a platelet transfusion?
Prophylaxis if platelets <10 If septic, abnormality of coagulation <20 Bleeding/procedure/surgery <50 Brain/eye/bleeding surgery <100
155
How does acute intermittent porphyria present?
Abdominal pain Neuropathies Constipation
156
How can a diagnosis of acute intermittent porphyria be made?
Elevated level of porphobilinogen on a spot urine test during acute attack
157
How is acute intermittent porphyria managed?
Mild: high doses of glucose Severe: haematin
158
What is acute intermittent porphyria?
Autosomal dominant disease resulting from defect in porphobilinogen deaminase so porphyrin precursors accumulate leading to neurological damage and psychiatric manifestations
159
What is idiopathic thrombocytopenic purpura?
Immune mediated reduction in platelet count | Antibodies directed against glycoprotein IIb/IIIa or Ib/V/IX complex
160
What is the most common type of Hodgkin's lymphoma?
Nodular sclerosing
161
Which factors are associated with a poor prognosis in Hodgkin's lymphoma?
``` Lymphocyte depleted type B symptoms: weight loss, fever, night sweats Age >45 Stage IV disease Haemoglobin <10.5 Lymphocyte count <600 Male Albumin <40 White cell count >15 ```
162
What are possible complications of CLL?
Hypogammaglobulinaemia leading to recurrent infections Warm autoimmune haemolytic anaemia Transformation to high grade lymphoma (richters transformation)
163
What is beta thalassemia major?
Absent synthesis of beta haemoglobin chains due to mutation in HBB gene on chromosome 11, autosomal recessive Inability to construct new beta chains leads to underproduction of HbA which leads to microcytic anaemia
164
What is HbF and HbA2?
Different forms of haemoglobin which are present in beta thalassemia HbF: alpha alpha gamma gamma HbA2: alpha alpha delta delta
165
What are the causes of DIC?
``` Severe sepsis and septic shock Malignancy Obstetric disease: eclampsia, abruption, amniotic fluid embolism Rheumatological disease Severe liver disease Transfusion reactions ```
166
What does a schilling test assess? What is it?
Absorption of vitamin B12 Following overnight fast, body injected with non labelled B12. This is followed by oral labelled B12 with cobalt 58, and a second dose with cobalt 57 bound to intrinsic factor Malabsorption if less than 10% labelled excreted in 24 hours Pernicious anaemia if elevated ratio of Co-57 to Co58
167
What is a typical finding of myelofibrosis on blood film?
Tear drop shaped cells
168
What is used in mild Von willebrand disease if there is evidence of bleeding?
DDAVP (desmopressin)
169
How is idiopathic thrombocytopenic purpura managed?
If symptomatic or platelet count below 20 then prednisolone | Splenectomy cures most cases
170
How is a bleed on warfarin managed?
If significant active bleeding then octaplex (prothrombin complex concentrate)
171
In which patients is pernicious anaemia most common?
``` Fair haired Elderly Women Blue eyes Autoimmune conditions: thyroid disease and vitiligo ```
172
How is pernicious anaemia managed?
IM vitamin B12/hydroxycobalamin
173
What is the leading cause of death in ITP?
Intracranial haemorrhage
174
What is the treatment choice for a patient with ITP and massive haemorrhage?
Platelet transfusion IV methylprednisolone IV immune globulin Splenectomy
175
In a patient presenting with an unprovoked DVT, other than commencing warfarin, what further actions are required?
``` Chest X-ray Bloods: FBC, calcium, LFTs Urinalysis If all normal: abdo pelvis CT and mammogram for woman in all patients over 40 Check antiphospholipid antibodies ```
176
What is haptoglobin?
Binds haemoglobin that has been released from erythrocytes | Can be used as measure of Haemolysis - levels will be decreased in intravascular
177
What are provocative factors for a sickle cell crisis?
``` Exercise Infection Fever Anxiety Hypoxia Changes in temperature ```
178
What is acute chest syndrome?
Vaso occlusive crisis of pulmonary vasculature seen in patients with sickle cell anaemia
179
What are complications of sickle cell?
``` Acute chest syndrome Pulmonary infarction Pneumonia Stroke Renal papillary necrosis Priapism Aseptic necrosis of bones and joints Digital infarction Leg ulcers ```
180
What can be given to induce release of Von willebrands factor from endothelial cells and therefore reduce bleed risk in theatre?
Desmopressin
181
What is the most common inherited thrombophilia?
Activated protein c resistance (factor V Leiden)
182
What are some inherited causes of hypercoagulable state?
``` Factor V Leiden Prothrombin gene mutation Anti thrombin deficiency Protein c and s deficiencies Elevated homocysteine Dysfibrinogenaemia Sickle cell disease ```
183
What are some causes of acquired hypercoagulable state?
``` Antiphospholipid syndrome Supplemental oestrogen use Heparin induced thrombocytopenia and thrombosis (HITT) Cancer Obesity Pregnancy ```
184
What is the function of activated protein c?
Along with cofactor protein s it inactivates factor Va and VIIIa
185
What is the mechanism of action of heparin?
Unfractioned - binds to antithrombin III to help inactivate thrombin LMWH - factor Xa inhibitor
186
What is waldenstroms macroglobulinaemia?
Lymphoplasmacytic lymphoma producing excess IgM Presents with bone marrow infiltration, splenomegaly and lymphadenopathy, raynauds, visual disturbance due to hyperviscosity
187
How is a diagnosis of hereditary spherocytosis made?
Osmotic fragility test
188
What is the management of hereditary spherocytosis?
Folate replacement | Splenectomy
189
Which factors are associated with increased bleeding risk when on anticoagulant drugs?
``` Poor INR control Hepatic or renal disease EtOH abuse Malignancy Anaemia Prior bleeding history Age Excessive fall risk Prior history of stroke Concomitant antiplatelets ```
190
Which indication for anticoagaultion has a target INR higher than 2.5?
Recurrence of VTE while on warfarin | Mechanical prosthetic heart valve
191
Why do warfarin and heparin need to overlap when treating acute venous thromboembolism with loading doses of warfarin?
Time delay in achieving an antithrombotic effect based on the elimination half-lives of the vitamin K dependent clotting factors Protein C: Warfarin is initially prothrombotic due to decreasing protein c levels due to short half life
192
What are the pharmacokinetics of warfarin?
Readily absorbed from the gastro-intestinal tract Plasma half-life is about 40 hours It is metabolised in the liver, and is excreted in the urine mainly as metabolites
193
What are some non haemorrhagic adverse effects of warfarin?
``` Rash Alopecia Nausea Diarrhoea Effects in utero: Congential abnormalities and foetal death if given to pregnant woman 6-14 weeks gestation ```
194
Which foods and drinks does warfarin interact with?
EtOH: Binge drinking Vitamin supplements: Vitamin K containing Diet: Foods rich in vitamin K eg broccoli, brussels sprouts, cabbage, kale, lettuce, spinach, watercress, and other green leafy vegetables
195
What colours are the different doses of warfarin?
0.5mg white 1mg brown 3mg blue 5mg pink
196
What baseline tests need doing before commencing warfarin?
FBC LFTs INR
197
Why is it important to ensure the blue top bottle is filled correctly for calculating an INR?
Underfilled citrate bottle will cause high INR result as it will cause high citrate levels Citrate binds calcium. Calcium is important for initiating clotting
198
What patient advice should be offered when starting warfarin?
Individual dose is adjusted to achieve desired level of anticoagulation Dose made up of tablets of different strengths and colours INR monitored regularly so must attend for checks Monitor for bleeding and know what to do if it happens Keep record safe and up to date Avoid medicines containing aspirin Don't start or stop new drugs including OTC without checking with dr or pharmacist first - huge number of interactions Avoid binge drinking Tell anyone involved in treating them that they are on warfarin
199
What does an oral anticoagulant patient pack contain?
Yellow Book (treatment record) Patient information Patient alert card
200
When should INR be checked after starting warfarin?
Day 5, 8 and 12
201
What are options to reverse warfarin?
Vitamin K: IV or PO | Prothrombin Complex Concentrate eg octaplex/beriplex: Factors II, IV, IX, X, protein c and s
202
How long does oral and IV vitamin K take to work?
Oral: Takes 24 hours to work IV: Takes 6-8 hours to work
203
What are different levels of thrombotic risk assessed for patients on warfarin before surgery? What does this mean for peri operative bridging therapy?
High risk: recent history of VTE (<3 months), or recurrent VTE on increased target INR (3.5) warfarin and severe prothrombotic disorder eg known Antiphospholipid Syndrome or Antithrombin Deficiency. Need therapeutic LMWH Moderate risk: history of recurrent VTE within last 3-12 months. Need prophylactic LMWH Low risk: majority of AF patients and patients with past history of single episode of VTE >12 months ago. No bridging required
204
What monitoring is required when a patient is on unfractionated heparin?
Need for regular APTTr and FBC every 6 hours
205
In which patients is routine monitoring required for LMWH? What monitoring is done?
``` Pregnancy Infancy <1 year old Renal failure Morbid obesity Active bleeding with no apparent cause Anti Xa levels ```
206
What are the pharmacokinetics of heparin?
LMWH: Rapidly and completed absorbed via S/C injection. Peak activity 1-4 hours post dose. Renal elimination primarily (half life 4-5 hours) although small degree of hepatic metabolism IV heparin: Elimination as above, Half life 30 mins – 2 hours
207
What are non bleeding adverse effects of heparin?
``` Allergic HITT Local skin reaction Hyperkalaemia Osteoporosis ```
208
What are some indications for therapeutic LMWH?
Treatment of VTE / peripheral vascular surgery Treatment of unstable angina and non-Q-wave myocardial infarction Treatment of acute ST-segment elevation myocardial infarction Prevention of extracorporeal thrombus formation during haemodialysis
209
When should a sample be taken to check for anti Xa levels?
4 hours post dose | 2 tubes required
210
What can be used to reverse heparin?
Protamine
211
What are contraindications to use of DOACs?
``` Heart valves Renal failure GFR <15 Strong CYP inhibitors eg systemic azoles HIV drugs PGP inhibitors: Amiodarone, clarithromycin, colchicine, omeprazole, nifedipine, sertraline, tamoxifen, verapamil ```
212
When should DOACs be stopped before surgery?
Minor surgery 24 hours Major 48 hours Renal failure 72 hours
213
What is the novel antidote for dabigatran? When can it be used?
Idarucizumab (Praxbind) Emergency major surgery (not to be used in elective or non-emergency cases) Life-threatening or uncontrolled bleeding
214
You are asked to see a 49 year old woman with a mechanical mitral heart valve on long term warfarin in the pre-op assessment clinic prior to laparascopic cholecystectomy. How would you manage her peri-operative anticoagulation?
Stop warfarin 5 days pre op Give bridging LMWH from 3 days pre op Last dose 24 hours before surgery
215
What are features of HIIT syndrome?
``` DVT PE MI Occlusion of limb arteries (possibly resulting in amputation) Cerebrovascular accidents (stroke,TIA) Skin necrosis End-organ damage (adrenal, bowel, spleen, gallbladder or hepatic infarction, renal failure) Death ```
216
24 year old woman with x2 previous DVTs on long term warfarin admitted c/o abdominal discomfort. Routine pregnancy test positive. What action needs taking?
Stop warfarin immediately and commence LMWH / liaise with obstetrics
217
76 year man, known atrial fibrillation on warfarin. Attends routine cardiology clinic. INR noted to be high (6.5). No bleeding apparent. What treatment is required?
PO vitamin K
218
78 year old man with inoperable lung carcinoma is.During admission develops a swollen, painful leg. Ultrasound confirms a popliteal DVT. How do you manage his initial anticoagulation?
Start LMWH monotherapy
219
What goes wrong that leads to leukaemogenesis?
``` Loss of cell-cycle control Autonomous proliferation Increased self-renewal Differentiation block Resistance to apoptosis: increased or decreased Dissemination Loss of tumour suppressor genes ```
220
What is the management of neutropenic sepsis?
Treat as neutropenic without waiting for FBC result Blood cultures Broad spectrum antibiotics within 60 minutes of presentation IV fluid resuscitation Get help
221
What living conditions are important when undergoing chemo for AML?
``` Filtered air No plants or flowers No unnecessary visitors Washed food – no salad or grapes or blackpepper Antifungal prophylaxis Mouthcare ```
222
What are indications for bone marrow biopsy?
Diagnostic: Abnormal FBC, Paraproteinaemia, Bone lesions in pelvis accessible by this route, Pyrexia of unknown origin, Isolated splenomegaly with diagnosis unclear from peripheral blood Staging: Hodgkin Lymphoma / Non Hodgkin Lymphoma Treatment response: Leukaemia, Myeloma, Lymphoma
223
What investigations needs to be done for multiple myeloma?
Protein electrophoresis serum/urine Bone marrow examination Skeletal survey
224
What causes rouleaux formation in blood film?
Plasma protein concentration high, raised ESR | Caused by: infections, myeloma, waldenstrons macroglobulinaemia, connective tissue disorders, cancer
225
What causes renal failure in myeloma?
``` Light chain deposition in kidney Hypercalcaemia Hyperuricaemia Dehydration Non-steroidal anti-inflammatories Plasma cell infiltration of kidney ```
226
How does the Philadelphia chromosome lead to CML?
Translocation causes constitutive activation of a tyrosine kinase at basis of the disease Constant activation of cell turnover
227
What is the management of CML?
Initial control of very high white cell count to prevent stroke – aspirin Drugs to control white cell count Leucopheresis Imatinib: inhibitor of the BCR-ABL tyrosine kinase inhibitor
228
What levels of haematocrit (PCV) are high?
PCV>0.60 – men | PCV>0.56 – women
229
Which mutation is found in association with primary polycythemia?
JAK2 Signals from EPO into cell Red cell production in absence of growth factor when mutated
230
What is management of primary polycythemia?
Reduce risk of cardiovascular events as result of increased abnormal red cell production: Venesections Aspirin Drugs to suppress bone marrow function
231
What are myeloproliferative disorders?
Clonal, pre-leukaemic Uncontrolled proliferation of one or more bone marrow lineages: Red cells – primary polycythaemia Platelets – essential thrombocythaemia White cells (myeloid) – chronic myeloid leukaemia Fibroblasts - myelofibrosis
232
What are malignant causes of hyperviscosity?
Paraprotein (IgM > IgA > IgG) High WCC (CML / AML > CLL) High red cell mass (polycythaemia) Raised platelet count (>1,000, myeloproliferative rather than reactive)
233
What are causes of splenomegaly?
Haematological: Chronic myeloid leukaemia, Myelofibrosis, Chronic lymphatic leukaemia, Acute lymphoblastic leukaemia, Lymphoma Infective: EBV, Chronic malaria, Visceral Leishmaniasis Liver: HCV / HBV with portal hypertension, Any cause cirrhosis with portal hypertension
234
What are indications for lymph node biopsy?
Generalised lymphadenopathy, FBC unhelpful Palpable cervical LN with mediastinal LN on CXR Isolated lymphadenopathy – no obvious pathology in anatomical region drained, (ENT: nasendoscopy NAD, FNA unhelpful) Regional lymphadenopathy with obvious primary pathology inaccessible to biopsy
235
What are some possible findings from lymph node biopsy?
``` Reactive Necrotic Granulomatous – TB, Sarcoid? HIV Metastatic Carcinoma Metastatic Melanoma Lymphoma ```
236
What are risks of chemo and radiotherapy?
``` Breast cancer Thyroid cancer Secondary leukaemia / myelodysplasia Infertility Other endocrine failure - early menopause Bones Cardiac damage (chemo + radiotherapy) ```
237
What are causes of anaemia?
``` Physiological Impaired production: BM failure, Dyserythropoesis- eg thalassaemia, Aplasia, leukaemia, Anaemia of chronic disease Reduced haematinics (Fe, Folate, B12) Increased breakdown (Haemolysis) Increased loss (Haemorrhage) Multifactorial ```
238
What are the stages of development of iron deficiency anaemia?
Normal Low iron stores: low ferritin Pre-clinical iron deficeiency: low MCV Clinical iron deficiency: low Hb
239
What are causes of iron deficiency anaemia in children?
Dietary: especially infants and toddlers, Fussy eaters, High milk intake, High requirements/prematurity Malabsorption Increased loss: GIT, menstruation
240
How should haemolytic anaemia be investigated?
Reticulocytes: increased LDH: high levels Haptoglobin: absent (intravascular haemolysis) Direct Coombs Test (autoimmune haemolysis, Looking for immunoglobulins on red cells) Blood Film for medical review Thalassemia and haemoglobinopathy screen as appropriate Further testing depending on results of initial tests
241
How can haemolytic anaemia present?
Anaemia Jaundice Splenomegaly Hyperbilirubinaemia
242
What are some causes of haemolytic anaemia?
Intrinsic: Membrane defects: hereditary spherocytosis Abnormal Hb: Sickle cell, Thalassaemia Enzyme defect: G6PD Extrinsic: Haemolytic Disease of newborn: Rh Dx and ABO incompatibility Drug induced HUS
243
How can you prevent megaloblastic crisis in hereditary spherocytosis?
Folate supplementation
244
What causes red cell aplasia in hereditary spherocytosis?
Parvovirus infection
245
Which populations are mainly affected by sickle cell disease?
African, Middle Eastern, Asian
246
What is sickle cell disease?
``` Amino acid substitution in Beta-globulin chain of haemoglobin Autosomal Recessive Normal (adult) Hb AA Carrier trait AS Sickle cell Diagnosis: Hb SS, SC, SD ```
247
How can sickle cell patients present?
Crisis: Pain- limbs, chest, abdomen, Aplastic, Sequestration
248
What are complications of sickle cell disease?
``` Anaemia Hb ~80 Splenic infarction – hyposplenism Infection Poor growth Leg ulcers, stroke ```
249
What is the management of sickle cell disease?
Prevention: antenatal diagnosis Prophylaxis: folate, antibiotics, avoid cold and dehydration Crisis: fluids, antibiotics, analgesia, exchange transfusion, Mobile auromated red cell exchange service Longer term management: Hydroxycarbamide, Bone marrow transplantation Experimental: gene therapy, anti P-selectin monoclonal antibody, Crizanlizumab
250
What is thalassaemia?
Due to reduced globin chain synthesis Normal: HbA (alpha 2 and beta 2) +small amounts of HbF and A2) Beta-thalassaemia: Genes on Chromosome 11, Thalassemia major– HbF (alpha 2 and gamma 2) and Hb A2 (alpha 2 and delta 2) – Thalassemia intermedia, Thalassemia minor Alpha-thalassemia: 2 alpha globin genes on each chromosome 16, Loss of 1 or 2 alpha genes: asymptomatic: alpha Thal Trait, Loss of 3 genes: HbH Disease, Loss of 4 genes: HbBarts Hydrops Foetalis
251
What is the management of beta thalassaemia?
``` Genetic counselling Regular blood transfusions Fe overload- complications: Liver, heart, pancreas, endocrinopathy Fe chelation BM Transplantation ```
252
What is the prognosis for acute leukaemia in children?
Prognosis >80% cured (age 2-10, female, WCC<50, no CNS involvement)
253
What are causes of bruising in children?
Trauma: Accidental, non-accidental Vascular: HSP, scurvy, ehlers danlos Platelets: ITP, leukaemia, Von willebrand Coagulation abnormality: haemorrhagic disease of newborn, haemophilia
254
What is Von willebrand factor?
Secreted by endothelium/platelets Carrier for FVIII:c Facilitates platelet adhesion
255
What tests and results would you expect in Von willebrand disease?
Prolonged bleeding time, PTT Low FVII:C, vWF, ristocetin cofactor assay vWF multimer analysis DNA tests
256
What are auer rods?
Material seen in cytoplasm of AML cells | Can also be seen in megaloblastic anaemia, leukaemia, iron deficiency anaemia hyposplenism - coeliac, thalassaemia
257
What are Howell jolly bodies?
``` Spherical blue black inclusions of red blood cells which represent residual nuclear fragments Commonly seen in asplenic patients Also severe haemolytic anaemia Pernicious anaemia Leukaemia Thalassaemia ```
258
What are pappenheimer bodies?
``` Violet staining granules in periphery of RBCs Sideroblastic anaemia Lead poisoning Thalassaemia Asplenic patients ```
259
With which condition are smudge cells associated?
CLL
260
How is a diagnosis of CLL confirmed?
Lymphocytosis Characteristic lymphocyte morphology (smudge cells) Immunophenotyping
261
What are features of polycythemia rubra Vera?
``` Hyperviscosity Pruritis (typically after hot bath) Splenomegaly Haemorrhage (2ary to abnormal platelets) Plethoric appearance HTN ```
262
What investigations should be done for polycythemia rubra Vera?
``` FBC Blood film JAK2 mutation Serum ferritin U and Es LFTs If JAK2 negative: red cell mass, arterial O2 sats, abdo USS, serum EPO, bone marrow aspirate and trephine, cytogenetic analysis, erythroid burst forming unit culture ```
263
What is the mechanism of action of DDAVP in Von willebrands disease?
Stimulates release of vWF from endothelial cells | Can increase levels long enough for surgery or dental procedures to be performed
264
Who should be screened for thrombophilia?
Unprovoked DVT or PE with first degree relative also had DVT or PE
265
What would you expect on a blood film in a patient with G6PD?
Heinz bodies
266
What is the diagnostic test for hereditary spherocytosis?
Osmotic fragility test
267
What is the inheritance pattern of G6PD deficiency?
X linked recessive
268
What are presenting features of hereditary spherocytosis?
``` Failure to thrive Jaundice Gallstones Splenomegaly Aplastic crisis precipitated by parvovirus MCHC elevated ```
269
What is management of hereditary spherocytosis?
Folate replacement | Splenectomy
270
What blood results would you expect to see with hypersplenism?
Pancytopenia | Raised reticulocytes
271
What is paroxysmal nocturnal haemoglobinuria?
Acquired clonal defect of red cells resulting in production of erythrocytes with increased susceptibility to lysis by complement Intermittent passage of bloody urine Prothrombotic tendency caused by complement induced platelet aggregation
272
What is protein S?
Vit K dependent anticoagulant which acts as non enzymatic co factor to activated protein c in proteolytic degradation of factor Va and VIIIa
273
What is factor V Leiden?
Hypercoagulability disorder resulting from variant of factor V which cannot be inactivated by activated protein c
274
What are features of fanconi anaemia?
``` Autosomal recessive Aplastic anaemia Increased risk of AML Neurological problems Short stature Cafe au lait spots ```
275
What are antibodies directed against in ITP?
Glycoprotein IIb/IIIa complex
276
What are indications for splenectomy?
Traumatic rupture ITP Spherocytosis
277
What prophylaxis do splenectomy patients need?
Vaccines 4 weeks before: pneumococcus, meningococcus, HIB | Lifelong Pen V
278
What investigations need to be done for myeloma?
Serum electrophoresis, serum free light chains Bone marrow examination Skeletal survey
279
What does vitamin K do?
Gamma carboxylation of factors II, VII, IX and X
280
Where does vitamin K come from?
50% from food (leafy vegetables, eggs) | 50% from bacteria in gut (why antibiotics affect warfarin control)
281
Why don't we use FFP in warfarin reversal any more?
High volume required in order to raise factor levels by small amounts
282
Which is the only DOAC that can be reversed?
Dabigatran (can be dialysed and has a Mab antidote)
283
What type of antibodies are anti A/B?
IgM (complement fix)
284
What is a delayed transfusion reaction?
Later onset, due to minor antibodies in the transfused blood Secondary immune response Not ABO incompatibility
285
What are important questions with regards to spine treatment for vertebral collapse in myeloma?
Have they had radiotherapy? | Have they had vertebroplasty?
286
What are markers of red cell breakdown?
Bilirubin Haptoglobin (absent) LDH
287
What are markers of increased red cell production?
Reticulocytosis
288
How do you investigate a cause of Haemolysis?
Blood film | Direct Coombs test (look for antibodies)
289
What are spherocytes?
Red cells more round as their membrane is lost | E.g. Autoimmune haemolytic anaemia, macrophages eat membrane
290
What are different causes of haemolytic anaemia?
Congenital: G6PD deficiency, sickle cell, hereditary spherocytosis Immune: autoimmune, alloimmune (ABO incompatibility) Non immune: intravascular, DIC, HUS/TTP, mechanical valve, accelerated HTN
291
What are causes of retinal haemorrhages?
Diabetes HTN Haematology: CML/AML hyperviscosity, paraproteinaemia IgM, polycythemia, raised platelet count
292
What are causes of splenomegaly?
Haem: CML, CLL, ALL, lymphoma Infective: EBV, chronic malaria, visceral leishmaniasis Liver: HCV/HBV with portal HTN, cirrhosis RA SLE
293
What is haptoglobin?
Binds free Hb with high affinity and so inhibits oxidative activity
294
What is the definition of a massive transfusion?
Entire patients blood volume/10 units of blood in 24 hours
295
What are early and late transfusion complications?
Early: acute haemolysis, bacterial contamination, allergic reaction, anaphylaxis, TRALI, fluid overload, non haemolytic febrile transfusion reaction Late: iron overload, graft vs host disease, post transfusion purpura, infection
296
What are complications of a massive transfusion?
Hypocalcaemia Hypothermia Hyperkalaemia Dilutional effect on other blood components eg thrombocytopenia
297
What are non megaloblastic causes of macrocytic anaemia?
``` Alcohol Liver disease Hypothyroidism Pregnancy Reticulocytosis ```
298
What drug is given to treat B12 deficiency?
Intramuscular hydroxycobalamin (B12)
299
What are causes of folate deficiency?
Poor diet: vegans Increased demand: pregnancy Malabsorption: coeliac disease Anti folate drugs: methotrexate
300
What might be examination findings in beta thalassaemia major?
``` Hepatosplenomegaly Frontal bossing Jaw enlargement Dental malocclusion Flow murmur Generalised pallor Conjunctival pallor ```
301
What is the common name for beta thalassaemia major?
Cooleys anaemia
302
What advice should be given to haemophiliacs?
Wear medic alert bracelet Avoid NSAIDs and IM injections Avoid contact sports and other activities

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