Haematology 4: Myeloproliferative Disorders

Question 1

What are the main differences between myeloproliferative and myelodysplastic disorders ?

Answer 1

Myelodysplastic syndromes have ineffective differentiation whereas in myeloproliferative disorders the cells are normal and fully differentiated.

Question 2

Give 4 examples of myeloproliferative disorders ?

Answer 2

Polycythaemia rubra Vera
Essential thropmbocythaemia
Idiopathic myelofibrosis
Idiopathic erythrocytosis

Question 3

List 4 signs/symptoms of Polycythaemia rubra Vera ?

Answer 3

Aquagenic pruritus- itch after having a warm bath
Gout
Engorged retinal veins
Erythromelalgia- Red painful extremities

Question 4

What levels would you expect in someone with Polycythaemia rubra Vera ?:

Hb :Low/High
Hct :Low/High
MCV: Low/High
EPO: Low/High

Answer 4

Hb: High
Hct: High
MCV: High
EPO: low (suppressed because of negative feedback)

Question 5

How is Polycythaemia rubra Vera diagnosed ?

Answer 5

Bone marrow biopsy- Increased cellularity, low EPO, Reticulin fibrosis

JAK2 V617F mutation is diagnostic

Question 6

What is pseudo polycythaemia ?

Answer 6

Reduced plasma volume in presence of a normal Hb conc.

Question 7

What causes Polycythaemia where EPO is raised ?

Answer 7

Hypoxia- High altitude
Renal disease
tumours

Question 8

What are the signs of Essential Thrombocythaemia ?

Answer 8

High platelet count
CVA
DVT/PE
Splenomegaly

Question 9

What are the signs of Chronic idiopathic myelofibrosis ?

Answer 9

FLAWS
anaemia
Thrombocytosis
Hepatosplenomegaly (massive, sites for extra medullary haematopoeisis)

Question 10

What is the characteristic finding of bone marrow aspiration in Chronic idiopathic myelofibrosis

Answer 10

Dry tap

Because of the fibrosis nothing comes out