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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
Key clinical feature in antiphospholipid syndrome?
predisposition to arterial and venous thromboses
26
Hallmark bloods finding in antiphospholipid syndrome?
paradoxical rise in APTT
27
Which autoimmune Abs are seen in antiphospholipid syndrome?
1. anticardiolipin antibodies 2. anti-beta2 glycoprotein I 3. lupus anticoagulant
28
What are the issues caused by antiphospholipid syndrome in pregnancy?
1. recurrent miscarriages 2. IUGR 3. pre-eclampsia 4. placental abruption
29
How is antiphospholipid syndrome treated in pregnancy?
low dose aspirin then LMWH when fetal heart is seen
30
What is the inheritance pattern of hereditary angioedema and how is it screened?
autosomal dominant. C2/4 levels between attacks
31
What are the main associations in each Type of cryoglobulinaemia?
Type 1 - Raynaud's, myeloma, WM Type 2 - hepatitis C Type 3 - RA, Sjogren's
32
Blood film finding in myelofibrosis?
Tear drop-like poikilocytes
33
Hallmark feature of Hodgkin’s lymphoma seen in bone marrow biopsy?
Reed-Sternberg cells
34
Which type of Hodgkin’s lymphoma is: 1. Most common 2. Best prognosis?
1. Nodular sclerosing 2. Lymphocyte dominant
35
What chromosomal translocation is associated with the following types of NHL? 1. Burkitt’s 2. Follicular 3. Mantle cell
1. T(8,14) 2. T(14:18) 3. T(11:14)
36
Key pathological feature in myelofibrosis?
hyperplasia of abnormal megakaryocytes
37
blood film finding in myelofibrosis?
tear-drop poikilocytes
38
mutation associated with both myelofibrosis and essential thrombocyto
CALR mutation
39
FBC finding in polycythaemia vera?
raised Hb + haemotocrit + neutrophils
40
bone barrow biopsy/bloodd film finding in AML?
myeloblasts with large nuclei +/- Auer rods
41
genetic abnormalities that carry poorer prognosis in AML?
deletion of chrososome 5/7
42
genetic abnormality seen in acute promyelocytic leukaemia?
t(15:17)
43
How does APL present classically?
DIC or thrombocytopenia
44
treatment specific to APL?
all-trans retinoic acid
45
genetic abnormality in CML?
T(9:22) in Philadelphia chromosome
46
blood film/FBC finding in CML?
leukocytosis, increased granulocytes
47
1st line treatment in CML and its mechanism?
imatinib, tyrosine kinase inhibitor
48
genetic abnormities that convey good and poor prognosis in CLL?
good prognosis: 13q deletion poor prognosis: 11q/17p deletion
49
what is Richter's transformation?
CD5+/CD23+ CLL B cells transforming into high-grde NHL
50
screening test for hereditary angioedema?
low C4 levels
51
key pathological feature and diagnostic test in hereditary angioedema?
low C1-esterase inhibitor
52
hallmark clinical feature in methaemglobulinaemia?
low SpO2 depsite normal pO2
53
treatment options for methaemoglobinuaemia?
ascorbic acid, methylthioninium chloride IV
54
Defining pathological feature of Waldenstrom's macroglobulinaemia?
monoclonal IgM paraproteins

MRCP Part 1 (6 decks)

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