GEORGE H. GOLDSMITH Jr., M.D.; ROBERT G. BAILY, M.D.; DOREEN B. BRETTLER, M.D.; WILLIAM R. DAVIDSON Jr., M.D.; JAMES O. BALLARD, M.D.; THOMAS E. DRISCOL, M.D.; JOSHUA M. GREENBERG, M.D.; CAROL K. KASPER, M.D.; PETER H. LEVINE, M.D.; OSCAR D. RATNOFF, M.D.
Five patients with classic hemophilia were found to have primary pulmonary hypertension, a disorder not previously recognized in this population. All patients had had their coagulation disorder treated for 10 years or more with self-administered lyophilized concentrates of factor VIII, and all had antibodies to human immunodeficiency virus (HIV). Primary pulmonary hypertension was confirmed by histologic means at autopsy in one patient and by lung biopsy findings in another. In the other three patients, the findings are in agreement with this diagnosis. No patient had underlying cardiac or pulmonary disease, or clinical or pathologic evidence of collagen-vascular disease, vasculitis, parasitic disorders, hemoglobinopathy, or exposure to anorexigenic agents. Whether the primary pulmonary hypertension was related to treatment with lyophilized factor VIII, or to the presence of antibodies to HIV, or both, is unknown.
GOLDSMITH GH, BAILY RG, BRETTLER DB, DAVIDSON WR, BALLARD JO, DRISCOL TE, et al. Primary Pulmonary Hypertension in Patients with Classic Hemophilia. Ann Intern Med. ;108:797–799. doi: 10.7326/0003-4819-108-6-797
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Published: Ann Intern Med. 1988;108(6):797-799.
Coagulopathies, Hematology/Oncology, Pulmonary Hypertension, Pulmonary/Critical Care.
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