Severe pulmonary vascular occlusive disease following bone marrow transplantation in Omenn syndrome
C Brückmann, W Lindner, R Roos… - European journal of …, 1991 - Springer
C Brückmann, W Lindner, R Roos, W Permanetter, RJ Haas, SG Haworth, BH Belohradsky
European journal of pediatrics, 1991•SpringerA 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 …
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 …
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.
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