Severe pulmonary vascular occlusive disease following bone marrow transplantation in Omenn syndrome.
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.
Brückmann
C
C
Universitäts-Kinderklinik München, Federal Republic of Germany.
Lindner
W
W
Roos
R
R
Permanetter
W
W
Haas
R J
RJ
Haworth
S G
SG
Belohradsky
B H
BH
D001157:Arterial Occlusive Diseases / Q000150:complications / Q000209:etiology*; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D004802:Eosinophilia / Q000276:immunology / Q000601:surgery; D006338:Heart Neoplasms / Q000276:immunology / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000601:surgery*; D007223:Infant; D008206:Lymphatic Diseases / Q000276:immunology / Q000601:surgery; D008297:Male; D011651:Pulmonary Artery / Q000473:pathology*