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 patients had idiopathic "warm" AHA (group I) and 18 had AHA associated with systemic diseases (group II). Complete response to splenectomy was defined as having normal hemoglobin and reticulocyte count lasting for at least 6 months without subsequent medical therapy. Subnormal but greater than 50% improvement 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, respectively. Nine of 11 (82%) evaluable patients with idiopathic AHA and 3 of 16 (19%) patients with associated disease achieved a complete response. Partial response was obtained in 2 (18%) and 6 (37%) patients in groups I and II, respectively. Both complete-response and overall-response rates were statistically different between two groups (P = 0.001 and 0.02). Postoperative courses of group I patients were uneventful except for one who developed a subphrenic abscess. Five patients in group II developed bacterial infections, which were mostly pneumonias. Our findings indicate that splenectomy is an effective treatment approach with low morbidity and mortality in patients with refractory idiopathic AHA. It should, however, be considered cautiously in AHA patients with underlying systemic diseases because of its decreased efficacy and increased surgical morbidity in this subgroup.