Thrombocytopenia is often associated with a variety of autoimmune disorders:
1. Systemic lupus erythematosus (SLE): Thrombocytopenia occurs in 15-25% of patients with SLE as a result of peripheral destruction of platelets. Treatment: corticosteroids, IVGG, immunosuppressive agents (e.g., azathioprine, cyclophosphamide), and vinca alkaloids. Splenectomy should be reserved for refractory cases and/or life-threatening hemorrhage.
2. Autoimmune lymphoproliferative syndrome (ALPS): This syndrome is characterized by massive lymphadenopathy, hepatosplenomegaly, hypergammaglobu-linemia, and autoimmune cytopenias including ITP. The pathogenesis is inherited defects in FAS and other genes that regulate lymphocyte apoptosis. The treatment is steroids.
3. Antiphospholipid antibody syndrome: Antiphospholipid antibodies enhance platelet activation. These patients have recurrent arterial or venous thrombi. The treatment is steroids and/or immunosuppressive agents.
4. Evans syndrome: Evans syndrome is the combination of autoimmune hemolytic anemia and thrombocytopenia and/or neutropenia. These patients have a poor response to steroids, IVIG, or splenectomy.
5. Other autoimmune processes: Hodgkin disease, non-Hodgkin lymphoma, juvenile rheumatoid arthritis, dermatomyositis, Graves' disease, Hashimoto thy-roiditis, myasthenia gravis, inflammatory bowel disease, sarcoidosis, and protein losing enteropathy may be associated with autoimmune thrombocy-topenia.
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