Essential thrombocythaemia - Myeloproliferative Disorders (MPD)

Essential thrombocythaemia

·        Clinical presentation:

o   Any age, usually older

o   Often asymptomatic 

o   Bleeding OR thrombosis (e.g. digital arteries ® necrotic toes)

o   Splenomegaly (2 – 3 cm) in 70%

·        Lab results:

o   ­Platelets, often > 1000 * 10E9

o   Morphology abnormal, normal plus large platelets

o   Platelet function studies abnormal

o   Hb normal or mild anaemia 

o   WBC normal or mild ­

o   Bone marrow: many megakaryocytes

·        Treatment:

o   Chemotherapy: hydroxyurea

o   Radioactive phosphorous: P32 – stored in bone so zaps marrow

o   Interferon

o   Prognosis: if platelets down then good, if not then bad