Internal Medicine

0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:17

Myeloma and Gammopathies Myeloproliferative Disorders 1037

Prognosis

■Dependent upon stage of disease; overall median survival is 3–4 years

for conventional therapy and 4–5 years posttransplant.

MYELOPROLIFERATIVE DISORDERS

ALISON R. MOLITERNO, MD

history & physical

■MPDs are acquired bone marrow stem cell disorders that share vary-

ing degrees of elevated peripheral cell counts, marrow fibrosis and

splenomegaly

■Many patients are identified by routine blood work and are otherwise

asymptomatic

■Symptoms and signs related to polycythemia include headaches,

itching especially after bathing, plethora, hypertension, gastroin-

testinal ulcers and venous or arterial thrombosis

■Symptoms related to splenomegaly include early satiety, left upper

quadrant fullness or pain

■Symptoms associated with high platelet counts include migraine

headaches, visual disturbances, erythromelalgia, easy bruising or

bleeding, transient ischemic attacks and venous or arterial throm-

bosis

tests

Molecular basis of these disorders is heterogeneous; diagnosis rests on

assembling clinical findings and laboratory tests

Basic Tests

■complete blood count with differential

■peripheral blood smear

■bone marrow exam to assess cellularity, fibrosis, karyotype

■peripheral blood assessment for JAK2 V617F mutation

Specific Tests

■Polycythemia vera

➣elevated red cell mass by isotope dilution

➣normal oxygen saturation > 92%

➣erythropoietin level – low or normal in PV

JAK2 V617F positive in 90% of PV

■Idiopathic myelofibrosis