0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:17
Myeloproliferative Disorders Myoclonus 1039➣consider allogeneic bone marrow transplant for younger patients
with progressive IMF
■Symptomatic thrombocytosis
➣bleeding or thrombosis may respond to platelet lowering agents,
such as anagrelide, hydroxyurea
➣low dose aspirin for erythromelalgia
follow-up
■PV – hemoglobin should be monitored monthly then q 3 months
once a stable regimen is achieved
■IMF–q3month CBC and physical
■ET–q6month CBC and physical
complications and prognosis
■Poor prognostic indicators for any of the MPDs include the develop-
ment of anemia in the setting of marrow fibrosis
■Thrombotic complications
➣Intra-abdominal thromboses (portal vein, hepatic vein) and
stroke are very high in patients with an uncontrolled red cell
mass
➣Thrombotic risk due to high platelet count alone is controversial
➣consider anti-platelet agent or lowering platelet count if history
of or strong risk factors for thrombosis are present
■Massive splenomegaly may result in splenic infarctions, portal
hypertension, varices, and worsening anemia
■Many patients with ET will progress to PV or IMF over years to
decades
■Poor prognostic indicators for IMF include hemoglobin <10 gm/dl,
age, percentage of blasts in periphery, presence of night sweats,
weight loss, fevers. Approximately 20% patients with IMF will pro-
gress to leukemia.MYOCLONUS
CHAD CHRISTINE, MD
history & physical
■Episodic uncontrolled focal or generalized jerks
■Occasionally assoc w/ seizure disorder
■May be spontaneous or stimulus dependent (action myoclonus)
■Sudden, rapid, twitch-like muscle contractions
■Distribution may be focal segmental or generalized
■May be assoc w/ infectious, degenerative or metabolic disease