Primary Immune Thrombocytopenia (ITP)
Pathophys: thrombocytopenia d/t auto Ab-mediated megakaryocyte destruction and ↓ plt production
Presentation: Presents w/ mucocutaneous bleeding; defined by isolated plt <100k, dx of exclusion; 10% have ITP + AIHA = Evans Syndrome; BMBx ↑ megakaryocytes (performed in >60yo to r/o MDS); anti-plt Ab testing not useful.
• Severe bleeding: plts, IVIG, methylpred 1g/d IV x3d (or dexamethasone 40mg/d x4d), consider romiplostim.
o If no response in plts, consider Amicar (0.1g/kg/30min→0.5-1g/hr) / tranexamic acid / activated FVII
• 1st line: dexamethasone 40 mg/d x4d (or prednisone 1 mg/kg/d PO x2-3wk → taper) +/- IVIG 1 g/kg/d IV x 2d
• 2nd line: rituximab, Nplate (romiplostim), danazol (older women), splenectomy (consider after 6mo-1y, rarely done)
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