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Severe pulmonary vascular occlusive disease following bone marrow transplantation in Omenn syndrome.

作者信息

Brückmann C, Lindner W, Roos R, Permanetter W, Haas R J, Haworth S G, Belohradsky B H

机构信息

Universitäts-Kinderklinik München, Federal Republic of Germany.

出版信息

Eur J Pediatr. 1991 Feb;150(4):242-5. doi: 10.1007/BF01955521.

DOI:10.1007/BF01955521
PMID:2029913
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.

摘要

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本文引用的文献

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6
Absence of lymphocyte ecto-5'-nucleotidase in infants with reticuloendotheliosis and eosinophilia (Omenn's syndrome).患有网状内皮组织增殖症和嗜酸性粒细胞增多症（奥门氏综合征）的婴儿缺乏淋巴细胞外5'-核苷酸酶。
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Hum Pathol. 1983 Jan;14(1):88-91. doi: 10.1016/s0046-8177(83)80052-5.