Autoimmune hemolytic anemia is a rare disease in childhood. Its course is variable and unpredictable. The optimal management particularly of the chronic form of the illness is not well defined. A retrospective study of cases occurring in children, excluding newborns, in the Czech Republic from 1998 to 2001 has been undertaken using a structured questionnaire. The aim of the study was to survey the state of diagnostics and management of this disease as well as to look for pertinent prognostic factors. Autoimmune hemolytic anemia was diagnosed in 32 children aged from 1.2 to 18.8 years (median: 7.6). There were 18 boys and 14 girls in this series (M:F = 1.3:1). Twenty-three patients had an acute form and 8 a chronic form of the disease. In 22 cases, the disease was idiopathic (primary) and in 10, it was secondary with an associated chronic disorder involving the immune system. The acute form was very often associated with a recent or concomitant infection, usually of viral origin, and affected mainly young children (median age: 4.6 years), with slightly more boys being afflicted (M:F = 1.3:1). By contrast, the chronic form was encountered rather in older children (median age: 12.6 years), equally affecting both sexes, and was more frequently associated with a chronic underlying disease (6/8 vs 4/23) and relatively more often presented as Evans’ syndrome (3/8 vs 3/23). Warm-reacting, cold-reacting, mixed and bithermal antibodies were found in 12, 9, 4 and 2 patients, respectively, and data on thermal optimum and amplitude were not available in 5 cases. One patient was not treated for the anemia, 31 received steroids, 8 additional therapy (of these, 4 were given immunosuppressive drugs), and 12 required transfusion. None underwent splenectomy. While all children with the acute form recovered, 7/8 with the chronic form have been living with evidence of disease.

        Key words: autoimmune hemolytic anemia, Evans’ syndrome, children, retrospective study