Haematology

Question 1

difference in hereditary spherocytosis and G6PD deficiency?

Answer 1

G6PD

• African and Mediterranean descent
• Heinz bodies
• Male only
• Measure enzyme activity of G6PD for gold standard diagnosis

Hereditary spherocytosis

• Northern european descent
• Spherocytes
• Male and female
• EMA binding test

Question 2

What are the causes of macrocytic anaemia?

Answer 2

Megaloblastic
- B12/Folate deficiency

Normoblastic

• alcohol
• liver disease
• hypothyroidism
• pregnancy
• reticulocytosis
• myelodysplasia
• drugs: cytotoxics

Question 3

What is warm autoimmune haemolytic anaemia associated with?

Answer 3

CLL - complication of

Question 4

Causes of Microcytic anaemia?

Answer 4

• iron-deficiency anaemia
• thalassaemia*
• congenital sideroblastic anaemia
• anaemia of chronic disease (more commonly a normocytic, normochromic picture)
• lead poisoning

Question 5

Mechanism of action of TXA?

Answer 5

Antifibrinolytic that reversibly binds to lysine receptor sites on plasminogen or plasmin. This prevents plasmin from binding to and degrading fibrin.

Question 6

Pappenheimer bodies is/are associated with?

Answer 6

Hyposplenism

Question 7

What is MGUS? Features?

Answer 7

Monoclonal gammopathy of undetermined significance
Common Paraproteinaemia, sometimes mistaked for myeloma

• Usually asymptomatic
• no bone pain or increased risk of infections
• around 10-30% of patients have a demyelinating neuropathy

Does not have immune dysfunction/lytic lesions like myeloma

Question 8

Causes of massive splenomegaly?

Answer 8

myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher's syndrome

Question 9

Complications of CLL?

Answer 9

• anaemia
• hypogammaglobulinaemia leading to recurrent infections
• warm autoimmune haemolytic anaemia in 10-15% of patients
• transformation to high-grade lymphoma (Richter’s transformation)

Question 10

Treatment of CML?

Answer 10

imatinib

Question 11

Target cells associated with what?

Answer 11

Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease

Question 12

‘Pencil’ poikilocytes associated with?

Answer 12

Iron deficiency anaemia

Question 13

What blood product is most likely to be contaminated?

Answer 13

Platelets

Question 14

What symptoms are associated with poor prognosis in hodgkins lymphoma?

Answer 14

weight loss > 10% in last 6 months
fever > 38ºC
night sweats

Question 15

DIC bloods?

Answer 15

DIC typical blood picture:
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products

Question 16

What is imatinib used for

Answer 16

CML

Question 17

Most common bleeding disorder?

Answer 17

Von willibrand

Question 18

Bleeding time bloods in Von willibrand?

Answer 18

Prolonged bleeding time
APTT may be prolonged
Factor VIII levels may be moderately reduced

Question 19

Treatment for post thrombotic syndrome?

Answer 19

Compression stockings

Question 20

Most common inherited thrombophilia (clot forming) condition?

Answer 20

Factor V leiden

Question 21

What are the exceptions for teeatment of VTE with a doac?

Answer 21

Antiphospholipid syndrome (LMWH then VKA)

Renal function <15 GFR (UH, LMWH, or LMWH then VKA)

Question 22

Paraproteins caused by what things?

Answer 22

MGUS or Multiple Myeloma.

Question 23

In sickle disease what is a sequestration crisis?

Answer 23

sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
associated with an increased reticulocyte count

Question 24

What are the bleeding times for haemophilia?

Answer 24

prolonged APTT

bleeding time, thrombin time, prothrombin time normal

Question 25

What is haemophilia, what is the most common type, mode of inheritance?

Answer 25

Clotting disorder. 90% caused by Haemophilia A X-linked.

Question 26

What is the cause of haemophilia A and B

Answer 26

Haemophilia A - Factor VIII deficiency | B - IX deficiency

Question 27

Management for ITP?

Answer 27

First-line treatment for ITP is oral prednisolone Could also use IVIG.

Question 28

Features of beta thalassaemia trait?

Answer 28

Mild hypochromic, microcytic anaemia - microcytosis is characteristically disproportionate to the anaemia. HbA2 raised (> 3.5%)

Question 29

Lymphocytes in lymphoma high or low?

Answer 29

Low

Question 30

Viral causes for neutropaenia?

Answer 30

HIV Epstein-Barr virus Hepatitis

Question 31

Iron deficiency anaemia transferrin results?

Answer 31

Transferrin is high (along with TIBC), T sats are low though

Question 32

Presentation of CML?

Answer 32

anaemia: lethargy weight loss and sweating are common splenomegaly may be marked → abdo discomfort an increase in granulocytes at different stages of maturation +/- thrombocytosis decreased leukocyte alkaline phosphatase may undergo blast transformation (AML in 80%, ALL in 20%)

Question 33

Is alpha thalassaemia associated with hb A2 % rise?

Answer 33

No.

Question 34

HIT onset time?

Answer 34

5 - 10 days post starting.

Question 35

Bleeding times in HIT?

Answer 35

Procoagulant so not prolonged

Question 36

Bleeding times in DIC? Other bloods?

Answer 36

Prolonged. Both PT and APTT. Others: - Low plts - Low fibrin - Raised fibrin degradation products

Question 37

Causes of DIC?

Answer 37

sepsis trauma obstetric complications e.g. aminiotic fluid embolism or hemolysis, elevated liver function tests, and low platelets (HELLP syndrome) malignancy

Question 38

Dabigatran reversal agent?

Answer 38

Idarucizumab

Question 39

if B12 and folate both low what would you treat first?

Answer 39

B12