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MRCP Part 1 > Haematology > Flashcards

Haematology Flashcards

1
Q

Key pathological feature in sideroblastic anaemia?

A

inability to form haem in mitochondria, causing iron deposition in the nucleus

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2
Q

Blood film and bone marrow biopsy finding in sideroblastic anaemia?

A

Blood film: basophillic stippling
Bone marrow biopsy: sideroblast in Prussian blue staining

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3
Q

Mainstay of sideroblastic anaemia management?

A

Supportive, vitamin B6 (pyridoxine) supplement

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4
Q

Blood film finding and investigation for diagnosis of sickle cell anaemia?

A

Blood film: sickle cells, target cells
Diagnosis: Hb electrophoresis

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5
Q

Mainstay of long-term management for sickle cell anaemia?

A

Hydroxycarbamide to increase production of HbF

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6
Q

What is the key pathological feature of G6PD deficiency and inheritance pattern?

A

Reduced glutathione leading to increased oxidative stress on RBCs. autosomal recessive

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7
Q

Blood film finding and investigation for diagnosis of G6PD deficiency?

A

Blood film: Heinz bodies
Diagnosis: G6PD enzyme assay

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8
Q

3 main drug groups that precipitate a G6PD deficiency crisis?

A
  1. anti-malarials e.g. primaquine
  2. ciprofloxacin
  3. sulphonamides
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9
Q

What is the key pathological feature of hereditary spherocytosis and inheritance pattern?

A

spherical RBCs trapped in spleen leading to haemolysis. autosomal dominant

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10
Q

Investiagtion for diagnosis for hereditary spherocytosis?

A

EMA binding

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11
Q

Management options for hereditary spherocytosis?

A

supportive Rx +/- transfusion, folate supplement, splenectomy

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12
Q

A bloods finding seen only in intravascular haemolysis?

A

decreased haptoglobin

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13
Q

A blood film finding seen only in microangiopathic haemolysis?

A

schistocytes

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14
Q

3 examples of intravascular and extravascular haemolysis?

A

Intravascular: mech fragmentation, G6PD, PND, cold autoimmune
Extravascular: haemoglobinopathies, spherocytosis, warm autoimmune

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15
Q

Diagnostic test for paroxysmal nocturnal haemoglobinuria

A

flow cytometry for low CD55/59

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16
Q

how does warm AIHA and cold AIHA differ in immunoglobulins, temp and site of haemolysis?

A

warm-cold
1. IgG-IgM
2. 37-4
3. extravascular-intravascular

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17
Q

5 clinical features of TTP?

A
  1. thrombocytopenia
  2. microangiopathic haemolysis
  3. pyrexia
  4. renal failure
  5. fluctuating neurology
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18
Q

Key pathological feature of TTP

A

platelet aggregation dueto large vWF multimers, as result of autoimmunne Ab against ADAMTS13 enzyme

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19
Q

blood findings in DIC?

A

low platelets
prolonged PT, APTT
low fibrinogen

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20
Q

most common inherited bleeding disorder and its inheritance pattern?

A

von Willebrand disease, autosomal dominant

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21
Q

Treatment for severe von Willebrand disease and what is its mechanisms

A

desmopressin - induces release of vWF

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22
Q

key pathological feature and inheritance pattern of Haemophilia A and B?

A

Haemophilia A: Factor VIII deficiency
Haemophilia B: Factor IX deficiency
X-linked recessive

23
Q

Inhibition of which coagulation factors are reduced in antithrombin III deficiency?

A

thrombin, Factor IX, Factor X

24
Q

what is the risk when starting warfarin for protein C deficiency?

A

warfarin-induced skin necrosis

25
Q

Key clinical feature in antiphospholipid syndrome?

A

predisposition to arterial and venous thromboses

26
Q

Hallmark bloods finding in antiphospholipid syndrome?

A

paradoxical rise in APTT

27
Q

Which autoimmune Abs are seen in antiphospholipid syndrome?

A
  1. anticardiolipin antibodies
  2. anti-beta2 glycoprotein I
  3. lupus anticoagulant
28
Q

What are the issues caused by antiphospholipid syndrome in pregnancy?

A
  1. recurrent miscarriages
  2. IUGR
  3. pre-eclampsia
  4. placental abruption
29
Q

How is antiphospholipid syndrome treated in pregnancy?

A

low dose aspirin then LMWH when fetal heart is seen

30
Q

What is the inheritance pattern of hereditary angioedema and how is it screened?

A

autosomal dominant. C2/4 levels between attacks

31
Q

What are the main associations in each Type of cryoglobulinaemia?

A

Type 1 - Raynaud’s, myeloma, WM
Type 2 - hepatitis C
Type 3 - RA, Sjogren’s

32
Q

Blood film finding in myelofibrosis?

A

Tear drop-like poikilocytes

33
Q

Hallmark feature of Hodgkin’s lymphoma seen in bone marrow biopsy?

A

Reed-Sternberg cells

34
Q

Which type of Hodgkin’s lymphoma is:
1. Most common
2. Best prognosis?

A
  1. Nodular sclerosing
  2. Lymphocyte dominant
35
Q

What chromosomal translocation is associated with the following types of NHL?
1. Burkitt’s
2. Follicular
3. Mantle cell

A
  1. T(8,14)
  2. T(14:18)
  3. T(11:14)
36
Q

Key pathological feature in myelofibrosis?

A

hyperplasia of abnormal megakaryocytes

37
Q

blood film finding in myelofibrosis?

A

tear-drop poikilocytes

38
Q

mutation associated with both myelofibrosis and essential thrombocyto

A

CALR mutation

39
Q

FBC finding in polycythaemia vera?

A

raised Hb + haemotocrit + neutrophils

40
Q

bone barrow biopsy/bloodd film finding in AML?

A

myeloblasts with large nuclei +/- Auer rods

41
Q

genetic abnormalities that carry poorer prognosis in AML?

A

deletion of chrososome 5/7

42
Q

genetic abnormality seen in acute promyelocytic leukaemia?

A

t(15:17)

43
Q

How does APL present classically?

A

DIC or thrombocytopenia

44
Q

treatment specific to APL?

A

all-trans retinoic acid

45
Q

genetic abnormality in CML?

A

T(9:22) in Philadelphia chromosome

46
Q

blood film/FBC finding in CML?

A

leukocytosis, increased granulocytes

47
Q

1st line treatment in CML and its mechanism?

A

imatinib, tyrosine kinase inhibitor

48
Q

genetic abnormities that convey good and poor prognosis in CLL?

A

good prognosis: 13q deletion
poor prognosis: 11q/17p deletion

49
Q

what is Richter’s transformation?

A

CD5+/CD23+ CLL B cells transforming into high-grde NHL

50
Q

screening test for hereditary angioedema?

A

low C4 levels

51
Q

key pathological feature and diagnostic test in hereditary angioedema?

A

low C1-esterase inhibitor

52
Q

hallmark clinical feature in methaemglobulinaemia?

A

low SpO2 depsite normal pO2

53
Q

treatment options for methaemoglobinuaemia?

A

ascorbic acid, methylthioninium chloride IV

54
Q

Defining pathological feature of Waldenstrom’s macroglobulinaemia?

A

monoclonal IgM paraproteins

MRCP Part 1 (6 decks)

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