Severe pulmonary vascular occlusive disease following bone marrow transplantation in Omenn syndrome |
| |
| Authors: | C. Brückmann W. Lindner R. Roos W. Permanetter R. J. Haas S. G. Haworth B. H. Belohradsky |
| |
| Affiliation: | (1) Universitäts-Kinderklinik München, Lindwurmstrasse 4, W-8000 München 2, Federal Republic of Germany;(2) Pathologisches Institut der Universität München, Thalkirchner Strasse 36, W-8000 München 2, Federal Republic of Germany;(3) Institute of Child Health, University of London, The Hospital For Sick Children, Great Ormond Street, WC1N 3JH London, UK |
| |
| Abstract: | ![]()
A 5-month-old infant presented with severe combined immunodeficiency disease, reticuloendotheliosis, and hypereosinophilia (Omenn syndrome) resulting in recurrent infections and endomyocardial disease. Bone marrow transplantation from an HLA-identical donor after chemotherapeutic conditioning led to both immunological and clinical recovery. Bone marrow transplantation, however, was followed by severe pulmonary occlusive disease. The patient gradually recovered while on increased inspiratory oxygen and the calcium channel blocker nifedipine. |
| |
| Keywords: | Omenn syndrome Hypereosinophilia Endomyocardial disease Bone marrow transplantation Pulmonary vascular obstruction |
| 本文献已被 SpringerLink 等数据库收录！ |
|
