Panniculitis, pancreatitis and very severe aplastic anemia in childhood: achallenge to treat

In childhood, aplastic anemia is a rare disease of unknown etiology. Beside s toxic effects, also an infectious or an autoimmune origin are discussed. We report on an 8-year-old boy with very severe aplastic anemia (VSAA) who developed pancreatitis together with panniculitis. Initially, active cytome galovirus (CMV) infection was thought to be possibly contributive. Ganciclo vir was tried resulting in clearance of CMV, but VSAA persisted. Two months after the onset of VSAA, oligosymptomatic pancreatitis was observed togeth er with the onset of severe febrile panniculitis, occurring with multiple p ainful enlarged subcutaneous infiltrates of up to 7 cm in diameter. Treatme nt according to the Severe Aplastic Anemia-94 (SAA-94) protocol consisting of glucocorticoids, cyclosporin A (CsA), anti-thymocyte globulin and granul ocyte colony-stimulating factor was instituted. Since this treatment did no t lead to remission after day 110, escalation of the CsA dose up to 8 mg/kg body weight was tried. This regimen resulted in complete recovery of panni culitis and symptoms of pancreatitis. Incomplete hematological remission wa s reached and, to date, the patient has not required transfusions for 6 mon ths. Because this boy suffered simultaneously from three rare disorders, wh ich all responded to intense immunosuppression, this observation may underl ine common autoimmune mechanisms of these distinct diseases.