Haematology 10 - Myeloproliferative Disorders

Question 1

define myeloproliferative disorder

Answer 1

form of bone marrow cancer

uncontrolled proliferation o a single stem cell type

all may progress to AML

Question 2

3 myeloproliferative disorders + overgrown cell line

Answer 2

primary myelofibrosis - haematopoietic stem cells
polycythaemia vera - erythroid cell line
essential thrombocythaemia - megakaryocytic cell line

Question 3

key mutation in myeloproliferative disorders

Answer 3

JAK2

Question 4

specific definition of myelofibrosis + 3 signs

Answer 4

scarring of the bone marrow

can be caused by all 3 myeloproliferative disorders

hepatomegaly
splenomegaly
portal hypertension
+/- cord compression (depends where it is)

Question 5

presenting features of myeloproliferative disorders

Answer 5

FLAWS

splenomegaly
portal HTN triad

vera - red face, clots, bath itch

ET - clots

Question 6

myelofibrosis blood film

Answer 6

teardrop shaped RBCs
/ dacrocytes

blasts

anisocytosis

dry tap

Question 7

managing primary myelofibrosis

Answer 7

monitor
SC transplant
supportive
chemotherapy

Question 8

managing polycyth vera

Answer 8

venesection
aspirin
chemotherapy

Question 9

managing essential thrombocythaemia

Answer 9

aspirin

chemotherpay