Myeloproliferative Disorders

Question 1

what are myeloproliferative disorders?

Answer 1

acquired disorders that have a clonal process

Question 2

where do the mutations occur?

Answer 2

stem cell level

Question 3

what are the common myeloproliferative disorders?

Answer 3

• polycythaemia vera
• CMML
• CLL
• essential thrombocythaemia
• myelofibrosis

Question 4

should these myeloproliferative disorders evolve, what condition results?

Answer 4

acute myeloid leukaemia

Question 5

what is the prognosis of the resultant AML?

Answer 5

bad prognosis due to resistance to treatment

Question 6

what happens to the marrow in myeloproliferative disorders?

Answer 6

the marrow is hyperproliferative, causes fibrotic changes, which can evolve into secondary myelofibrosis

Question 7

which mutation is found in most of the polycythaemia vera cases?

Answer 7

JAK-2 mutation

Question 8

which mutation is commonly found in CLL?

Answer 8

BCR oncogene

Question 9

how do you distinguish between true or relative polycythaemia?

Answer 9

• HCT - the value rarely exceeds more than 55% in relative polycythaemia
• red cell mass

Question 10

features of primary polycythaemia

Answer 10

• aquagenic pruritis
• splenomegaly
• multiple lineages are affected
• JAK-2 mutation

Question 11

what conditions are people with polycythaemia vera prone to?

Answer 11

• myocardial infarction
• duodenal ulcer
• venous thrombosis
• Budd-Chiari syndrome

Question 12

management of polycythaemia

Answer 12

• venesection (brings down the Hb)

- hydroxyurea (reduces the WCC and platelet count)

Question 13

what is the cause of primary polycythaemia?

Answer 13

inappropriate EPO production

Question 14

how do you treat polycythaemia vera?

Answer 14

cytotoxic drugs

Question 15

causes of secondary polycythaemia?

Answer 15

• appropriate EPO production

- ectopic EPO production

Question 16

how do you treat polycythaemia with appropriate EPO production?

Answer 16

• no cytotoxics because the marrow is working fine

- venesection

Question 17

how do you treat polycythaemia with ectopic EPO production?

Answer 17

these are normally caused by EPO-releasing carcinoma; treat the underlying tumour

Question 18

which cell types does CMML affect?

Answer 18

• neutrophils
• monocytes
  (granulomatous type)

Question 19

potential clues pointing towards CMML

Answer 19

• cancer treated with chemo/radio
• splenomegaly
• rash on the legs (dysplastic monocyte deposition)

Question 20

treatment for CMML

Answer 20

• hydroxyurea
• azocitidine
• bone marrow transplant

Question 21

causes of reactive thrombocytosis

Answer 21

• infection
• blood loss
• iron deficiency anaemia
• inflammation
• carcinoma
• post surgery
• tissue damage

Question 22

features of a myeloproliferative neoplasm

Answer 22

• skyhigh thrombocytosis

- normal inflammatory markers

Question 23

causes of pancytopenia?

Answer 23

• decreased production

- increased consumption

Question 24

what can decrease production of cells in pancytopenia?

Answer 24

• infiltration
• aplasia
• myelodysplasia

Question 25

what can increase consumption of cells in pancytopenia?

Answer 25

• splenomegaly
• autoimmune
• sepsis

Question 26

how can you distinguish between a decreased production or increased consumption pancytopenia?

Answer 26

marrow test for infiltrative, abnormal or normal marrow

Question 27

what is a leucoerythroblastic picture?

Answer 27

white and red cell precursors in the peripheral blood

Question 28

causes of a dry aspiration

Answer 28

• empty marrow
• fibrotic marrow
• hypercellular marrow

Question 29

what is a trephine biopsy?

Answer 29

bone marrow biopsy

Question 30

features of a myelofibrosis

Answer 30

• tear drop erythrocytes
• dry aspirate
• hepatomegaly
• leucoerythroblastic picture