Haematology 4 - Chronic myeloproliferative neoplasms Flashcards Preview

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Flashcards in Haematology 4 - Chronic myeloproliferative neoplasms Deck (26)
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1

What is the target of imatinib?

Mutated tyrosine kinase (by BCR-ABL gene)

2

What is the normal role of janus kinases?

They activate the STAT pathway, which promotes cell growth and replication

3

How does JAK2 mutation cause uncontrollable replication?

No longer need growth factor to activate

4

In polycythaemia vera, what else will be abnormal on the FBC other than red cell count?

Pronounced thrombocytophilia and slight granulocytophilia

5

Recall some symptoms of polycythaemia vera

Due to hyperviscosity:
- Headaches
- visual disturbances
- dyspnoea
Due to increased histamine release:
- peptic ulcer
- aquagenic pruritis

6

Recall four clinical findings in polycythaemia vera

Plethora
Erythromelalgia (red, painful extremities)
Gout
Retinal vein engorgement

7

What is the expected level of erythropoietin in polycythaemia vera?

Low

8

What mutation is present in all patients with polycythaemia vera?

JAK2

9

How should pseudopolychthaemia and polycythaemia vera be differentiated?

Isotype dilution method

10

Recall 3 causes of increased EPO

Hypoxia
Uterine myoma
Renal cancer

11

Recall 3 causes of pseudopolycythaemia

Alcohol
Obesity
Diuretics

12

Recall 3 ways in which polycythaemia vera can be treated

1. Venesection
2. Cytoreductive therapy
3. Aspirin to reduce thrombosis risk

13

What is idiopathic erythrocytosis?

An isolated erythrocytosis with low EPO, where JAK2 V617 mutation is absent (although JAK mutation in exon 12 may be present)

14

How should idiopathic erythrocytosis be treated?

Venesection only

15

How is essential thrombocytothaemia defined?

Chronic myeloproliferative neoplasm with a sustained thrombocytosis > 600 x 10^9/L

16

Which age group is most at risk of essential thrombocytothaemia?

Bimodal:
Small peak at 30y (M=F)
Larger peak at 55y (F>M)

17

What are the 2 most likely presentations for patients with essential thrombocytothaemia?

Thrombosis
or bleeding?? paradoxically

18

Is there a JAK2 mutation in essential thrombocytothaemia?

Yes in 50%

19

What is the best treatment for essential thrombocytothaemia?

Hydroxycarbamide

20

Which condition is "tear drop poikilocytosis" pathognemonic of?

Myelofibrosis

21

Which cells are hyperproliferating in myelofibrosis?

Mostly megakaryocytes and granulocytes

22

Recall 2 signs of myelofibrosis

Massive spleen
Anaemia

23

What is the best treatment for myelofibrosis?

Ruxolotinib - a JAK2 inhibitor

24

Recall 3 bad prognostic indicators in CLL

LDH raised
CD38+
11q23 deletion

25

Recall 3 good prognostic indicators in CLL

Hypermutated Ig gene
Low ZAP-70 expression
13q14 deletion

26

What is the 1st line treatment for CLL with a p53 deletion?

Ibrutinib

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