High-dose methylprednisolone is effective in the management of acute graft-versus-host disease in severe combined immune deficiency

To the Editor: Severe combined immune deficiency (SCID) is a heterogeneous group of inherited diseases characterized by significantly impaired immunity leading to death in infancy unless treated with bone marrow transplantation (BMT).1 When successful, BMT provides full immune reconstitution and long-term survival. Typical first-line treatment includes a regimen of 2 mg/kg/d MP.7 However, a prospective study on MP doses of 2 mg/kg/d failed to show a significant advantage of this dose regimen.8 In contrast, the use of higher doses of 20 mg/kg showed a 43% response rate in aGvHD.9 We studied the use of high-dose MP pulse in patients with SCID after matched unrelated donor BMT who had aGvHD of grade II or higher, which emerged in spite of aGvHD prophylaxis and was not tamed by standard 2 mg/kg MP. Sixteen patients who developed aGvHD after receiving MUD BMT for SCID between 1996 and 2006 were included in this study (Table I).