Haematology Flashcards

Question

What is the presentation of acute leukaemia?

Answer 1

Thrombocytopenic bleeding (Purpura and mucosal membrane bleeding). Petechial bleeding that is non blanching. - Infection because of neutropenia (predominantly bacterial and fungal) e.g cellulitis - Bone marrow failure - there is a complete replacement of bone marrow with blast cells. These cells are undifferentiated.

Answer 2

Blood count : lymphocytosis (over 5000 per mm3), thrombocytopenia Bone marrow is over 30% lymphocytes Loss of immunoglobulin production Haemolytic anaemia Smudge cells on blood film Immunophenotyping

Answer 3

G6PD enzyme assay

Answer 4

Death is usually due to bone marrow failure (haemorrhage, anaemia, or infection), transformation to acute leukaemia, portal or pulmonary hypertension, heart failure, cachexia, or myeloid metaplasia with organ failure.

Answer 5

- Anaemia (SOB, fatigue, pallor) - Thrombocytopenia (bruising, petechiae, epistaxis) - Neutropenia (bacterial infections, pneumonia, sepsis) Neoplastc infiltration: - Bone marrow (bone pain) - Thymus (palpable mass/airway compression) - Liver and spleen (hepatosplenomegaly) - Lymphadenopathy - Headaches, vomitting, nerve palsies, nuchal rigidity **Can also cause gum swelling which is classic for acute myeloblastic leukaemia**

Answer 6

Painless and typically has a rubbery feeling. If it were metastatic cancer from the lung it would typically feel very hard.

Answer 7

Treatment of rejection: Corticosteroids Anti-thymocyte globulin Plasma exchange Prevention of graft rejection: ABO matching Tissue typing (class 1 and 2 HLA) Prophylactic immunosuppression Immunosuppression: Corticosteroids (prednisolone) Calcineurin inhibitors (tacrolimus) - blocks interleukin 2 gene transcription Antiproliferatives (MMF) (mycophenolate mofetil) - these prevent lymphocyte proliferation

Answer 8

Menorrhagia Dyspepsia PR bleeding Symptoms of malabsorption (diarrhoea, weight loss) Jaundice Splenomegaly

Answer 9

Massive haemorrhage Bone marrow failure Prophylaxis for surgery Cardiopulmonary bypass

Answer 10

mis-sense mutation is that activated factor V (a clotting factor) is inactivated 10 times more slowly by activated protein C than normal

Answer 11

Acute rejection is mediated by cell and antibody defences. Cellular infiltration of graft by Tc cells, B - cells, NK cells and Macrophages. Causes endothelial inflammation and parenchymal damage.

Answer 12

Characteristic symptom is a burning sensation in the hands Thrombosis (venous or arterial) and haemorrhage can be seen. Symptoms of arterial and venous thromboses, digital ischaemia, gout, headache Mild splenomegaly

Answer 13

Immediate rejection = within minutes Acute rejection = less than 6 months Chronic rejection = more than 6 months

Answer 14

Immune is mosty extravascular Non-immune is mostly intravascular

Answer 15

Megakaryocyte proliferation results in an overproduction of platelets.

Answer 16

Autoimmune Drugs CLL

Answer 17

↓ G6PD → ↓ glutathione → increased red cell susceptibility to oxidative stress

Answer 18

Leukoerythroblastosis and splenomegaly Splenomegaly happens as a result of extra medullary haematopoeisis Trephine biopsy shows an excess of megakaryocytes, increased reticulin and fibrous tissue replacement Presence of a JAK2 mutation supports the diagnosis

Answer 19

Acute onset of fever, chills, dyspnoea, tachypnoea, tachycardia, hypotension, hypoxaemia and noncardiogenic bilateral pulmonary oedema leading to respiratory failure during or within 6 hours of transfusion. (shock picture)

Answer 20

CD19, 20 and 23 and CD5 positive CD19 and 20 are common markers to which we target therapy

Answer 21

Prognosis: Adults with ALL complete remission rate 78–91% leukaemia-free survival at 5y 30–35% Children with ALL 5y overall survival ~90% Poor risk patients (slow response to induction or Philadelphia positive) 5y OS 45% (most children survive, half of adults survive)

Answer 22

Acute respiratory distress Tachycardia Raised blood pressure Acute worsening pulmonary oedema on CXR Evidence of positive fluid balance Orthopnea **(shortness of breath, peripheral and pulmonary oedema, high blood pressure)**

Answer 23

Monoclonal proliferation of mature functionally abnormal B cells, these mature B lymphocytes accumulate in the bone marrow - physical suppression, prevents maturation

Answer 24

Painful criris Stroke Gallstones Chest crisis: - Chest pain - Fever - Worsening hypoxia

Answer 25

Von willebrand factor is a carrier molecule of factor 8

Answer 26

Defects in phagocyte function Complement deficiencies Absence or polymorphisms in pathogen recognition receptors

Answer 27

Caused by a C1 inhibitor deficiency Autosomal dominant

Answer 28

_Low risk for thrombosis_ Antiplatelet drugs (aspirin, anagrelide) _High risk for thrombosis_ Hydroxyurea, interferon-alpha Folate to prevent deficiency Ruxolitinib is a JAK2 inhibitor that has recently been licensed for use Haematopoietic stem cell transplant is the only treatment option with a potential for cure.

Answer 29

Treatment for haemolytic anaemia Support marrow function –Folic acid Correct cause –Immunosuppression if autoimmune * Steroids * Treat trigger eg.CLL, Lymphoma –Remove site of red cell destruction •Splenectomy –Treat sepsis, leaky prosthetic valve, malignancy etc. if intravascular Consider transfusion Causes for haemolysis: autoimmune - give steroids / immunosuppressants DRUGS CLL - treat CHAD Infections - treat sepsis (so basically, give steroids if autoimmune, treat any infections and cancers)

Answer 30

Megaloblastic = vitamin B12 and folate Normoblastic = * Alcohol * Liver disease * Hypohyroidism * Pregnancy * Reticulocytosis * Myelodysplasia * Cytotoxic drugs

Answer 31

Tyrosine kinase inhibitors Says to not give blood - will exacerbate hyperviscosity (I think this is in respect to tumour lysis syndrome)

Answer 32

Lab results: - Target cells - Reticulocytosis - Increased WBC Evidence of haemolysis (increased unconjugated bilirubin, increased lactate dehydrogenase, decreased haptoglobin) **Haemoglobin electrophoresis** = definitive diagosis

Answer 33

Blood count - increased WBC, increased lymphoblasts Bone marrow smear (hypercellular bone marrow, lymphoblast domination) Immunophenotyping - Terminal deoxynucleotidyl transferase (TdT) is a marker found in premature T and pre-mature B lymphoblasts that is used to diagnose acute lymphoblastic leukemia.

Answer 34

Unusual or opportunistic infections often combined with failure to thrive pneumocystis jirovecii CMV

Answer 35

Point mutation in the beta globin gene Produces haemaglobin S (HbS) - these cells containing HbS tend to crescent chapes when they are deoxygenated They have one normal and one abnormal beta globin chain

Answer 36

Antibodies against type B blood

Answer 37

Most common microdeletion syndrome Incidence is higher in those with down’s syndrome 2nd most common cause of developmental delay and major congenital heart disease

Answer 38

Combination chemotherapy (ABVD) +/- radiotherapy Monoclonal antibodies (anti-CD30) Immunotherapy (checkpoint inhibitors) Use of PET scan to assess response to treatment and to limit use of radiotherapy Rituximab an be used as well

Answer 39

Composed of T cell precursors or B cell precursors These cells are stuck in the blast phase (undifferentiated) Divide uncontrollably - take up a lot of space and nutrients in the bone marrow - cytopenias (anaemia, thombocytopenias, leukopenias)

Answer 40

High potassium High phosphate Low calcium High uric acid High urea and nitrogen containing compounds

Answer 41

Type 1 = quantitive deficiency (80% of patients) Type 2 = qualititive deficiency Type 3 = severe (complete) deficiency

Answer 42

Small molecular weight molecules can pass through the glomerulus - bence jones protein in the urine - causes proximal tubular necrosis Cast nephropathy Nephrocalcinosis as a result of hypercalciuria Free light chains deposit in the kidneys, heart and other organs - causing AL amyloidosis

Answer 43

O RhD neg red cells (AB plasma)

Answer 44

It is X linked recessive so Males Common in people from mediterranean and africa

Answer 45

hydroxyurea (hydroxycarbamide) is widely used to reduce the platelet count Anagrelide interferon-α is also used in younger patients low-dose aspirin may be used to reduce the thrombotic risk

Answer 46

Abnormal Ig secretion (hypogammaglobulinaemia, autoimmunity) Richter syndrome- can progress to aggressive lymphoma (large B cell lymphoma)

Answer 47

General features due to reduced oxygen delivery to tissues: Tiredness/pallor Breathlessness Swelling of ankles Dizziness Chest pain Potential History: –Dyspepsia GI bleeding –Other bleeding, eg menorrhagia –Diet (NB children and elderly) Increased requirement - pregnancy **Malabsorption** from gastrectomy and coeliacs Atrophic tongue Angular Chelitis Hypochromic, microcytic cells Koilonychia B12/folate deficiency = anaemia, neurological symptoms (subacute combined degeneration of the cord in B12 deficiency.) Megaloblastic anaemia: “Lemon yellow” tinge –Bilirubin, LDH –Red cells friable

Answer 48

Low haemoglobin Raised WCC (this is incontrast to myeloma which has leukopenia) Low platelets Bone marrow (90% B - Lymphoblasts)

Answer 49

Incidence peaks in the 6th decade ## Footnote hyperviscosity (stroke, vascular occlusion, thrombosis, TIA) **pruritus**, typically after a hot bath **splenomegaly** haemorrhage (secondary to abnormal platelet function) **plethoric appearance** hypertension in a third of patients Symptoms of hyperviscosity include loss of concentration, headaches, dizziness, blackouts, pruritis and epistaxis

Answer 50

Aplastic crisis (temporary arrest of erythropoeisis, can be caused by parovirus B19) Vaso-occlusion, tissue ischaemia and infarction Daytylitis Hyposplenism means increase risk of infection MI VTE Dysrhythmias Acute chest syndrome (fever, chest pain, hypoxemia, wheezing, cough, respiratory distress) Pulmonary hypertension Osteoporosis Proliferative retinopathy Multi-organ failure Priapism

Answer 51

Tests for auto immune haemolytic anaemia Used to detect antibodies or compliment proteins attached to red cells f the red cells then agglutinate, the direct Coombs test is positive, a visual indication that antibodies or complement proteins are bound to the surface of red blood cells and may be causing destruction of those cells.

Answer 52

Impairment of DNA synthesis, cell cycle cannot progress from G2 growth stage to the mitosis stage - continued cell growth without division

Answer 53

Massive splenomegaly Fatigue Erythromelalgia constitutional symptoms (weight loss, night sweats, low-grade fever, cachexia, fatigue, and pruritus)

Answer 54

Binet staging

Answer 55

EBV HIV Collagenosis Lymphoma Drug induced (quinine)

Answer 56

A = factor 8 B = factor 9

Answer 57

Iron deficiency anaemia Thalassaemia Congenital sideroblastic anaemia Lead poisoning Anaemia of chronic disease (although this is more commonly a normocytic picture)

Answer 58

Antibodies against A and B

Answer 59

Proliferation of myeloid haematopoetic stem cells Overproduction of megakaryocytes in the bone marrow leading to bone marrow fibrosis

Answer 60

Burkitt (very aggressive) Diffuse large B cell (aggressive) - most common subtype of lymphoma Extranodal marginal zone MALT - slow growth Follicular (slow growth) - 2nd most common subtype Low grade lymphoma - indolent, often asymptomatic, responds to chemotherapy but is itself incurable HIgh grade - aggressive and fast growing, can be cured

Answer 61

Neoplastic monoclonal proliferation of myelogenous stem cells (myeloblasts) in bone marrow Immature myeloblasts accumulate in bone marrow → physical suppression → prevents maturation

Answer 62

Defect in the cytoskeleton in red blood cells Red blood cell survival is reduced due to destruction by the spleen

Answer 63

Pretest probability scoring Wells score Geneva score Laboratory testing if pretest probability low D-dimer Imaging Doppler US - compression ultrasonography, contrast venography, CT venography, MRA DVT thrombosed vein enlarged, non-compressible, echogenic material might be seen Ventilation perfusion scan CT pulmonary angiogram

Answer 64

Full cross match ABO / RhD specific Choose antigen negative blood if there are alloantibodies

Answer 65

Infection Fava beans Drugs (NSAIDs, aspirin, nitrofurantoin, quinine)

Answer 66

Folate/B12 + bone marrow

Answer 67

Increasing age Pregnancy Surgery Obesity Systemic disease Family history Tissue trauma Immobility Hormonal therapy (COCP/HRT) COCP = combined oral contraceptive pill Antipsychotics

Answer 68

Treatment for megaloblastic anaemia: Replace vitamin B12 deficiency –B12 intramuscular injection –Loading dose then 3 monthly maintenance Folate deficiency –Oral folate replacement –Ensure B12 normal if neuropathic symptoms

Answer 69

Screening for factor V Leiden is not recommended, even after a venous thromboembolism. The logic behind this is that a previous thromboembolism itself is a risk factor for further events and this should dictate specific management in the future, rather than the particular thrombophilia identified.

Answer 70

Correct the deficiency - Oral iron usually sufficient IV iron if intolerant of oral Blood transfusion rarely indicated Correct the cause - Diet Ulcer therapy Gynae interventions Surgery

Answer 71

CHAD Infections Lymphoma

Answer 72

* Coagulation factor replacement FVIII/IX * Now almost entirely **recombinant** products * DDAVP (desmopressin) (this can be used in patients with haemophilia A, von willebrand disease and thrombocytopenia, cannot be used for haemophilia type B. The drug works by increasing the amount of von willebrand factor and factor 8. * Tranexamic Acid * Emphasis on prophylaxis in severe haemophilia * Gene therapy? * Splint * Physiotherapy * Analgesia * Synovectomy * Joint replacement Treatment is designed to get the person to the point where they are no longer severely haemophilic (we want to make sure they no longer bleed spontaneously which is the definition of severe)

Answer 73

Promotes platelet adhesion to damaged endothelium

Answer 74

PTT = intrinsic pathway (intrinsic pathway contains factor 8 and 9) PT = extrinsic pathway

Answer 75

tranexamic acid for mild bleeding desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells factor VIII concentrate VW factor concentrate

Answer 76

Median survival: Stage A - same as matched controls Stage B - around 8 years Stage C = around 6 years

Answer 77

Clinical features ## Footnote bone disease: bone pain, osteoporosis + pathological fractures (typically vertebral), osteolytic lesions lethargy infection hypercalcaemia renal failure other features: amyloidosis e.g. Macroglossia, carpal tunnel syndrome; neuropathy; hyperviscosity (hyperviscosity syndrome caused by malignant cells producing lots of paraproteins) Bleeding tendancy (retinal, oral, nasal or cutaneous)

Answer 78

Septicaemia Malignancy (acute myeloid leukaemia can cause DIC) Eclampsia

Answer 79

lymphadenopathy (75%) - painless, non-tender, asymmetrical systemic (25%): weight loss, pruritus, night sweats, fever (Pel-Ebstein) alcohol pain in HL normocytic anaemia, eosinophilia LDH raised

Answer 80

Its a neoplastic disorder of plasma proteins that usually results in the excessive production of a single type of immunoglobulin (paraprotein) Peaks in 7th decade More common in black people than white.

Answer 81

Recurrent episodes of painless, non-pitting, non-pruritis, non-erythematous swellings Swellings can be: - Subcutaneous - Intestinal - Oropharynx

Answer 82

ABO / RhD specific

Answer 83

Thromolysis Antiplatelet/anticoagulant drugs

Answer 84

Ankyrin Alpha spectrin Beta spectrin Band 3 Protein 4.2

Answer 85

It has a bimodal age distributions being most common in the third and seventh decades

Answer 86

Cytokine receptor defects are the most common cause - most commonly adenosine deaminase deficiency - low levels of this enyme result in a decline of T cells and B cells

Answer 87

Chronic kidney disease Haemolytic anaemia Aplastic anaemia Anaemia of chronic disease

Answer 88

Chemotherapy Immunomodulators such as thalidomide Proteasome inhibitors, IMiDs, monoclonal antibodies Bisphosphonate therapy (Zoledronic acid) - these prevent bone loss Radiotherapy Steroids Surgery Pinning of long bones; decompression of spinal cord Autologous stem cell transplant Antibiotics to treat infections

Answer 89

Iron deficiency You don't give iron because that will cause a stroke

Answer 90

Absence of beta chains Chromosome 11

Answer 91

Thirst Constipation Solomnence Confusion Nausea

Answer 92

Blood count shows increased granulocytes (basophils, eosinophils and neutrohpils) Bone marrow biopsy shows hypercellularity (cells of myeloid cell line / precursors) FISH/PCR for karyotypic analysis: BCR-ABL 1 gene mapping Small chromosome 22 = philidelphia chromosome. Small chromosome is the result of balanced translocation with chromosome 9. You are left with a small 22 and a BCR - ABL on 22 - this then results in an increased amount of ABL.

Answer 93

_Chemotherapy_ ## Footnote Consolidation therapy Maintenance treatment Stem cel transplant if high risk Large portion of care is to ensure children continue to grow and develop - keep up with school etc CNS directed therapy (chemotherapy doesn’t penetrate well and it often spreads to the CNS) - give medicines intrathecally by lumbar tap or lumbar puncture Maintenance treatment for 18 months _Bone marrow transplant in those that are likely to relapse_ Newer therapies: 1) Bispecifc T-cell engagers (BiTe molecules) – e.g. Blinatumumab - draws t cells to cancerous cells to destroy the cancer 2) CAR (chimeric antigen receptor T-cells) Patient/ healthy 3rd party T-cells harvested Transfected to express a specific T-cell receptor expressed on leukaemia cells (CD19) Expanded in vitro Re-infused into patient CAR - T cells have risk of **cytokine release syndrome** - signs include fever, hypotension and dyspnoea

Answer 94

Folci acid Transfusion Splenectomy

Answer 95

Febrile non-haemolytic transfusion reaction TACO TRALI Allergic Transfusion assocaited graft vs host disease Prion disease such as CJD

Answer 96

Venessection to keep the haematocrit below 0.45 and 0.43 in women Aspirin Hydroxycarbamide Ruxolitinib - JAK 2 inhibitor Interferon alpha

Answer 97

Need to do serum ferritin, reduced serum ferritin means iron deficiency anaemia If male you might want to do a GI endoscopy to check for bleeding

Answer 98

Blood count will show leukocytosis and anaemia Bone marrow aspirate (over 20% of the cells withh be myeloblasts) Myeloblasts containing Auer rods Others: - Immunophenotyping of leukaemic blasts (remember that acute lymphoblastic will contain terminal deoxynucleotidyl transferase) - CSF examination if there are symptoms

Answer 99

- IgG, IgA paraproteins - these are produced by mature plasma cells after isotype switching

Answer 100

Presence of reed-sternberg cells Giant B cells with bilobed nuclei that have prominent eosinophilic inclusions

Answer 101

Failure to thrive Hepatosplenomegaly Bony deformities Growth retardation

Answer 102

bone marrow failure or AML HSTC is the only curative option

Answer 103

Abnormal surface or physiological activator

Answer 104

Life long prophylaxis –Vaccination –Penicillin (and malarial) prophylaxis –Folic acid Acute Events –Hydration –Oxygenation –Prompt treatment of infection –Analgaesia * Opiates * NSAIDs Blood transfusion –Episodic and chronic –Alloimmunisation –Iron overload Disease modifying drugs –**Hydroxycarbamide** - this increases the amount of fetal haemoglobin in the blood and so therefore decreases the number of crisis' - L glutamine - duces sickling Bone marrow transplantation Gene therapy Treatment for painful crisis: •Severe pain- often requires opiates - Analgesia should be given within 30 mins of presentation - Effective analgesia by 1hour - Avoid pethidine * Hydration * Oxygen * Consider antibiotics No routine role for transfusion Treatment for a chest crisis: Respiratory Support Antibiotics IV Fluids Analgaesia Transfusion – top up or exchange target HbS \<30%

Answer 105

Repeated blood transfusions To prevent iron overloading you have to use a chelation therapy s/c desferroxamine is gold standard - oral alternative is deferasirox Bone marrow transplant = curative

Answer 106

Cerebrovascular or coronary events occur in up to 60% of patients. The disease may convert to another myeloproliferative disorder with about 25% developing acute leukaemia or myelofibrosis

Answer 107

Involved in adherence = GP 1b and GP 1a Involved in activation = ADP and COX Involved in aggregation = thromboxane A2 Involved in coagulation = scramblase

Answer 108

Correct anaemia which may cause organ damage if left uncorrected To improve quality of life in a patient with otherwise uncorrectable anaemia To prepare a patient for surgery or to speed up recovery To reverse damage caused by patients own red cells

Answer 109

Recurrent bacterial infections of the upper and or lower respiratory tract S.pneumoniae H.influenzae

Answer 110

DAT - not an immune reaction therefore there is a negative result No tests necessary if there is a family history, typica clinical features and typical lab findings (spherocytes, raised MCHC, raised reticulocytes) Cryohaemolysis test and EMA binding

Answer 111

Raised haemoglobin concentration and haematocrit Tendancy to also have a raised white cell count and platelet count Raised uric acid since there is an increased cell turnover Increase in red cell mass when the blood volume is measured Presence of JAK2 mutation - in JAK 2 negative cases you have to rule out secondary causes of erythrocytosis

Answer 112

* Haemarthrosis * Muscle haematoma * CNS bleeding * Retroperitoneal bleeding * Post surgical bleeding Complications can include: Synovitis Chronic Haemophilic Arthropathy Neurovascular compression (compartment syndromes) Other sequelae of bleeding (Stroke)

Answer 113

Protein C Protein S TFPI (tissue factor pathway inhibitor) Antithrombin These confine the clot to the area of tissue damage

Answer 114

Both are treated with combination chemotherapy Both are treated with combination chemotherapy – typically anti-CD20 monoclonal antibody + chemo CD20 monoclonal antibody is rituximab

Answer 115

Behaves like a platelet disorder i.e. epistaxis and menorrhagia are common whilst haemoarthroses and muscle haematomas are rare

Answer 116

Defective B cell function Absence of mature B cells

Answer 117

Pernicious anaemia, caused by gastric / ileal disease Antibodies against intrinsic factor (diagnostic) Gastric parietal cells (less specific) Malabsorption of dietary B 12 Symptoms / signs take 1-2 years to develop produced by the parietal cells of the stomach. It is necessary for the absorption of vitamin B12 later on in the ileum of the small intestine.

Answer 118

Fever Drenching sweats, loss of weight, prutitis, fatigue B symptoms: fever, drenching sweats, 10% non-deliberate weight loss, fatigue, pruritis High grade makes you feel ill very quickly - not usually found by accident Low grade grows slowly and are often found by accident

Answer 119

History and examination Blood cultures -Hickman line & peripheral CXR Throat swab & other clinical sites of infection (if UTI do MSSU) Sputum if productive FBC, renal and liver function, coagulation screen

Answer 120

Neonatal jaundice (like HS) Drug, borad bean or infection precipitated jaundice and anaemia (haemolysis) Intravascular haemolysis (HS is extravascular) Splenomegaly Pigment gallstones

Answer 121

Chromosomal Changes: Deletion of the long arm of chromosome 13 (del 13q) is the most common abnormality, being seen in around 50% of patients. It is associated with a good prognosis Deletions of part of the short arm of chromosome 17 (del 17p) are seen in around 5-10% of patients and are associated with a poor prognosis

Answer 122

Anaemia Splenomegaly, often massive Weight loss Hyperleukostasis - fundal haemorrhage and venous congestion, altered consciousness, respiratory failure. Also known as tumour lysis syndrome Gout from a high cell turnover

Answer 123

Other innate components Cytokines and antibodies cause myointimal proliferation in the arteries - blocks blood vessels and leads to ischaemia and fibrosis

Answer 124

Greater than 600 x 10^9

Answer 125

Fever with no localising signs Single reading of \>38.50C or 380C on two readings one hour apart Rigors Chest infection/ pneumonia Skin sepsis - cellulitis Urinary tract infection Septic shock

Answer 126

JAK2V617F in 95%

Answer 127

Neoplastic monoclonal proliferation of ature granulocytes/precursors Mature granulocytes accumulate in the bone marrow causing physical suppression which prevents maturation Chronic myeloid leukaemia is assocaited with the philidelphia chromosome - t(9,22) BCR-ABL1 fusion - chimeric protein with strong tyrosine kinase activity (in over 90% of individuals)

Answer 128

CATCH 22 Cardiac abnormalities Abnormal facies Thymic hypoplasia Cleft palate Hypocalcaemia Also includes: - Learning difficulties

Answer 129

Microcytic

Answer 130

B/T cell tumours No Reed-sternberg cells

Answer 131

Most presentation is between 6 months and 2 years. The baby starts walking and then all of a sudden stops walking, because they have had a bleed in their joint and don’t want to walk on it. Don’t jump to non accidental injury until you rule out bleeding disorder.

Answer 132

No antibodies

Answer 133

Bone marrow plasma cells

Answer 134

Want to perform a reticulocyte count and establish if the bone marrow is working correctly

Answer 135

Recurrent RTI's during infancy Low T cell numbers Low IgA and IgM Reduced antibody response Autoimmune phenomena: -Anaemia/thrombocytopenia Juvenile chronic arthritis (JIA) Raynauds Thyroid disease

Answer 136

Most common leukaemia in adults Agent Orange exposure - Anaemia (SOB, fatigue, pallor) - Thrombocytopenia (bruising, petechiae, epistaxis) - Neutropenia (bacterial infections, pneumonia, sepsis) Neoplastc infiltration: - Bone marrow (bone pain) - Thymus (palpable mass/airway compression) - Liver and spleen (hepatosplenomegaly) - Lymphadenopathy - Headaches, vomitting, nerve palsies, nuchal rigidity

Answer 137

``` Prolonged APTT (activated partial thromboplastin time) Normal PT (prothrombin time) ```

Answer 138

6 units of red cells 4 units of fresh frozen plasma 1 units of platelets

Answer 139

Progressive bone marrow failure Massive lymphadenopathy Progressive splenomegaly Lymphocyte doubling time \<6 months or \>50% increase over 2 months Systemic symptoms Autoimmune cytopenias

Answer 140

Drugs for DVT: Anticoagulants: LMWH Warfarin DOACs Thrombolysis only in selected cases: Massive PE E.g streptokinase, tissue plasminogen activator

Answer 141

Transfusion reactions

Answer 142

Impaired T cell function or the absence of normal T cells

Answer 143

Anti-leukaemic chemotherapy (to achieve consolidation and remission) Danorubicin Cytosine arabinoside Gemtuzumab Ozgamicin CPX351 **All trans retinoic acid** and arsenic trioxide in low risk acute promyelocytic leukaemia (chemo free) high cure rate - 90% Targeted Treatment: Midostaurin in FLT3 mutated AML Targeted antibodies: Gemtuzumab Ozogamicin anti-CD33 with Calicheomycin (Mylotarg) - (think of Milo playing tig) Targeted small molecules Midostaurin, Tyrosine Kinase Inhibitor including inhibiting FLT3 New delivery systems CPX -351 - old fashioned chemotherapy but in a lipid molecule

Answer 144

Arterial thrombus = white clot - platelets and fibrin, results in ischaemia and infarction, principally secondary to atherosclerosis. ‘Red thrombus’~fibrin and red cells Results in back pressure Principally due to stasis and hypercoagulability

Answer 145

IgM paraproteins Biopsy (lymph node, bone marrow) CT scan Bone marrow aspirate and trephine

Answer 146

2-3 week history of bone marrow failure or bone/joint pain. Anaemia Tiredness Fatigue Infection Bone marrow can necrose because it is full of rapidly dividing cells Bone pain Limp or hip pain (red flag symptom in children) Osmosis: - Anaemia (SOB, fatigue, pallor) - Thrombocytopenia (bruising, petechiae, epistaxis) - Neutropenia (bacterial infections, pneumonia, sepsis) Neoplastc infiltration: - Bone marrow (bone pain) - Thymus (palpable mass/airway compression) - Liver and spleen (hepatosplenomegaly) - Lymphadenopathy - Headaches, vomitting, nerve palsies, nuchal rigidity

Answer 147

Stasis Hypercoagubility Vessel damage Stasis = bed rest, travel Hypercoagulability = pregnancy, trauma Vessel damage = atherosclerosis

Answer 148

HSCT - haematopoeitic stem cell transplantation

Answer 149

B cell or T cell lineage Grade of disease (high grade or low grade) Histological features of the disease

Answer 150

FNHTR = anti-pyretics Urticaria = Antihistamines

Answer 151

TACO is frequently confused with TRALI as a key feature of both is pulmonary oedema and it is possible for these complications to occur concurrently. Hypertension is a constant feature in TACO whereas it is infrequent and transient in TRALI.

Answer 152

* Petechia (Venous pressure is higher in the leg than anywhere else – more likely to have petechiae) * Ecchymosis (discolouration of the skin resulting from bleeding underneath, typically caused by bruising) * Mucosal Bleeding * Rare CNS bleeding

Answer 153

Blast cell count has to be greater than 20% for the diagnosis Less than 20% may indicate myelodysplastic syndrome

Answer 154

Hein Bodies

Answer 155

Recurrent infections Diarrhoea, malabsorption and failure to thrive Oral candidiasis with prominent thrush Complications in clude susceptibility to severe infections such as: andida albicans, pseudomonas, pneumocystiss jiroveci, cytomegalovirus, varicella

Answer 156

JAK2 mutation in around 50% of patients Can also be associated with the CALR mutation in around 25% of individuals

Answer 157

Decreased production: Marrow failure Aplasia Infiltration Increased consumption Immune ITP Non immune DIC (caused by septicaemia, malignancy and eclampsia) Hypersplenism

Answer 158

Stop transfusion immediately and follow other steps for managing suspected transfusion reactions. Place the patient in an upright position and treat symptoms with oxygen, diuretics and other cardiac failure therapy.

Answer 159

DAT test will tell you wether or not it is immune or non-immune. DAT positive = immune, DAT negative = not immune. Of the immune reactions the positive types can be further subdivided into warm-autoantibody, cold antibody and allo antibody.

Answer 160

Viral infection: HIV, HBV, HCV Development of inhibatory antibodies for factor 8 (up to 10-15% of patients with haemophilia A) DDAVP SE include MI and hyponatraemia

Haematology Flashcards

(188 cards)