Comparative response to splenectomy in coombs-positive autoimmune hemolytic anemia with or without associated disease

We reviewed our experience in 30 patients with direct Coombs-positive (DAT) autoimmune hemolytic anemia (AHA) who underwent splenectomy. Twelve patie nts had idiopathic "warm" AHA (group I) and 18 had AHA associated with syst emic diseases (group II). Complete response to splenectomy was defined as h aving normal hemoglobin and reticulocyte count lasting for at least 6 month s without subsequent medical therapy. Subnormal but greater than 50% improv ement in these parameters with or without medical therapy was considered to be a partial response. Median age was 64 (23-81) in group I and 68 (23-76) in group II. Median follow-up duration was 18 and 10.9 months, respectivel y. Nine of 11 (82%) evaluable patients with idiopathic AHA and 3 of 16 (19% ) patients with associated disease achieved a complete response, Partial re sponse was obtained in 2 (18%) and 6 (37%) patients in groups I and II, res pectively. Both complete-response and overall-response rates were statistic ally different between two groups (P = 0.001 and 0.02). Postoperative cours es of group I patients were uneventful except for one who developed a subph renic abscess. Five patients in group II developed bacterial infections, wh ich were mostly pneumonias. Our findings indicate that splenectomy is an ef fective treatment approach with low morbidity and mortality in patients wit h refractory idiopathic AHA. It should, however, be considered cautiously i n AHA patients with underlying systemic diseases because of its decreased e fficacy and increased surgical morbidity in this subgroup. Am. J. Hematol, 61:98-102, 1999. (C) 1999 Wiley-Liss, Inc.