Prolonged remission after splenectomy for refractory Evans syndrome--a case report and literature review.
We describe a patient with Evans syndrome (autoimmune hemolytic anemia and autoimmune thrombocytopenia) who was refractory to steroids and intravenous immunoglobulin. She responded to splenectomy and has remained in clinical remission for 3 years. In the majority of cases, splenectomy rarely induces a durable remission but it may be beneficial in a small group of patients, hence should be considered as alternative therapy in the management of these patients.
Hamidah
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Department of Pediatrics, Faculty of Medicine UKM, Hospital Universiti Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, Cheras, 56000 Kuala Lumpur, Malaysia. hamidaha@mail.hukm.ukm.my
Thambidorai
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CR
Jamal
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D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000628:therapy*; D001803:Blood Transfusion; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D011241:Prednisone / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000628:therapy*; D012008:Recurrence; D012074:Remission Induction; D013156:Splenectomy; D013577:Syndrome
D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D011241:Prednisone