Myeloproliferative Disorders

Question 1

MD

Answer 1

cancer of bone marrow, proliferation of a mature stem cell

Question 2

what are the examples of MD

Answer 2

myelofibrosis
polycythaemia vera
essential thrombocytosis

Question 3

myelofibrosis

Answer 3

malignant proliferation of mature myeloid stem cell causing fibrosis of the bone marrow

causes:
polycythaemia vera
essential thrombocytosis

signs:
B symptoms
anaemia
splenomegaly 
pancytopenia 

investigations:
tear drops on blood film
dry tap on bone aspiration
bone biopsy

management:
treat symptomatically

Question 4

polycythaemia vera

Answer 4

increased concentration of haemocrit, RBC due to increase production of RBC from the marrow

causes:
primary
secondary - COPD, pregnancy, apnoea

signs:
splenomegaly
burning sensation on hands
itch after contact with warm water

investigations:
FBC

EPO - low in primary cause, high in secondary cause

management:
venesection
hydroxycarbamide 
aspirin as prophylaxis
reduce RBC

complications:
MI, stroke, DVT, VTE signs

Question 5

essential thrombocytosis

Answer 5

elevated platelet levels

causes:
myelofibrosis

signs:
burning sensation 
normal RBC levels 
elevated platelet levels
DVT, VTE

management:
hydroxycarbamide
aspirin as prophylaxis

Question 6

what is a common complications of these disorders

Answer 6

Acute Myeloid Leukaemia

Question 7

what mutation is common with polycythaemia vera

Answer 7

JAK2