Myelofibrosis - Myeloproliferative Disorders (MPD)

Myelofibrosis

·        Clinical presentation:

o   Old age

o   Preceding polycythaemia in 30%

o   Anaemia

o   Slow onset, weight loss, night sweats – insidious 

o   Massive hepatosplenomegaly. Large spleen ® pressure on splenic blood supply ® infarction ® pain 

·        Lab results:

o   Hb low

o   WBC high early, low late

o   Platelets high early, low late

o   Leucoerythroblastosis

o   Tear drop red cells

o   LAP (leucocyte alkaline phosphatase)

o   Philadelphia chromosome –ive

o   Fibrosis of bone marrow 

·        Treatment: Mainly supportive: observation, low does chemotherapy, splenectomy (symptomatic effect only)

·        Prognosis:

o   Mean survival 3 – 4 years, may become transfusion dependent

o   10% develop AML