warm agglutinin disease

warm agglutinin disease

Immunology An autoimmune hemolytic syndrome caused by IgG antibodies; 40% are 2º to underlying conditions–eg, neoplasia–eg, CLL, ovarian teratoma, connective tissue disease–eg, SLE, progressive systemic sclerosis, rheumatoid arthritis, ulcerative colitis, etc Clinical Brisk hemolysis, Sx of anemia–ie, pallor, fatigue, exertional dyspnea, vertigo, palpitations, jaundice, splenomegaly Lab Anemia, positive antiglobin–Coombs' test, spherocytes, schistocytes, erythrophagocytosis; BM–erythroid hyperplasia, ±underlying lymphoproliferative disorder Treatment Transfusions; steroids ↓ hemolysis in2⁄3 of Pts; 20% achieve complete remission at >20 mg/day;2⁄3 respond to splenectomy, but may relapse; modalities with varying degrees of failure include immunosuppressants and plasmapheresis Prognosis 73% 10-yr survival. Cf Cold agglutinin disease.