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 lon...

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Veröffentlicht in:Journal of allergy and clinical immunology 2008-12, Vol.122 (6), p.1215-1216
Hauptverfasser: Somech, Raz, MD, Kavadas, Fotini D., MD, Atkinson, Adelle, MD, Grunebaum, Eyal, MD, Roifman, Chaim M., MD, FRCPC
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container_end_page 1216
container_issue 6
container_start_page 1215
container_title Journal of allergy and clinical immunology
container_volume 122
creator Somech, Raz, MD
Kavadas, Fotini D., MD
Atkinson, Adelle, MD
Grunebaum, Eyal, MD
Roifman, Chaim M., MD, FRCPC
description 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).
doi_str_mv 10.1016/j.jaci.2008.06.023
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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. 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source MEDLINE; Elsevier ScienceDirect Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
subjects Acute Disease
Age
Allergy and Immunology
Anti-Inflammatory Agents - administration & dosage
Blood pressure
Bone marrow
Bone Marrow Transplantation
Disease-Free Survival
Female
Graft vs Host Disease - drug therapy
Graft vs Host Disease - genetics
Graft vs Host Disease - mortality
Humans
Infant
Infant, Newborn
Male
Medical treatment
Methylprednisolone - administration & dosage
Patients
Retrospective Studies
Severe Combined Immunodeficiency - genetics
Severe Combined Immunodeficiency - mortality
Severe Combined Immunodeficiency - therapy
Transplants & implants
title High-dose methylprednisolone is effective in the management of acute graft-versus-host disease in severe combined immune deficiency
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