Info

🌱 來自: platelet disorders

Primary immune thrombocytopenic purpura (ITP)

• Isolated thrombocytopenia due to immune plt destruction (auto-Ab to plts) & ↓ production (auto-Ab to megakaryocytes) without precipitant

• Clinical manifestations: insidious onset of mucocutaneous bleeding;

• ♀:♂ = 3:1

• Diagnosis of exclusion (r/o 2° ITP); no robust clinical or lab parameters, but typically:

  • CBC: isolated ↓ plt (<100,000/µL);
  • 10% have ITP + AIHA = Evans syndrome
  • Peripheral smear: large platelets (not specific), r/o pseudothrombocytopenia
  • BM bx: ↑ megakaryocytes, nl cellularity. Consider if other CBC or smear abnl or diagnostic uncertainty (Blood 2011;117:4910).

• ✓ HBSAg & anti-HBc prior to rituximab (and before IVIg, which could alter results)

• Treatment: rarely indicated if plt >50,000/µL unless bleeding, trauma/surgery, anticoag.

Treatment of Primary ITP in Adults