LETTER
Primary Pulmonary Hypertension in Association with Human T-Cell Lymphotropic Virus Type I in a Hemophiliac
Toru Suzuki, MD
1 September 1995 | Volume 123 Issue 5 | Page 394
TO THE EDITOR:
I describe the first known case of primary pulmonary hypertension (PPH) in a hemophiliac with human T-cell lymphotropic virus type I (HTLV-I).
A 44-year-old Japanese man with hemophilia A had HTLV-I infection caused by blood transfusions. He experienced aggravating dyspnea on exertion. The physical examination, chest radiograph, and echocardiographic findings suggested pulmonary hypertension. Cardiac catheterization confirmed pulmonary hypertension with near systemic pulmonary artery pressures. Pulmonary capillary wedge pressures were not elevated. No secondary cause of pulmonary hypertension could be found despite extensive testing. The diagnosis of PPH was thus established. Despite transient episodes of aggravation of symptoms, the patient's medical course overall has been favorable.
Primary pulmonary hypertension has previously been described in hemophiliacs [1] and nonhemophiliacs [2-4] with human immunodeficiency virus (HIV) infection; this has suggested a causal relation between HIV and PPH. A lack of reports of HTLV-I infection with similar clinical profiles, however, represented a missing link in the support of a causative role common to the retroviruses. This report describes the first known case of PPH in a hemophiliac with HTLV-I infection and no coinfection with HIV. Recent reports have shown that a serum factor produced by T cells infected by the human retroviruses, HIV and HTLV-I, stimulates and supports growth of vascular endothelial cells, a critical process in the pathogenesis of PPH and the most probable pathogenetic mechanism in our patient [5]. Interestingly, this factor, which has yet to be characterized, also stimulates and supports the development of Kaposi sarcoma, which is commonly associated with HIV and has also been reported in HTLV-I infection [5]. This case of PPH with HTLV-1 suggests that a factor common to the human retroviruses may play a critical role in the proliferative process.
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Author and Article Information
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University of Tokyo; Tokyo 153 Japan
1. Goldsmith GH JR., Bailey, RG, Brettler DB, et al. Primary pulmonary hypertension patients with classic hemophilia. Ann Intern. Med. 1988; 108:797-9.
2. Mette SA, Palvesky HI, Pietra GG, et al. Primary pulmonary hypertension in association with human immunodeficiency virus infection: a possiable viral etiology for some forms of hypertensive pulmonary arteriopathy. Am Rev Respir Dis. 1992; 145:1196-200.
3. Coplan NL, Shimony RY, Ioachim HL, et al. Primary pulmonary hypertension associated with human immunodeficiency viral infection. Am J Med. 1990; 89:96-9.
4. Speich R, Jenni R, Opravil M, Pfab M, Russi FW. Primary pulmonary hypertension in HIV infection. Chest. 1991; 100: 1268-7.
5. Nakamura S, Salahuddin SZ, Biberfeld P, et al. Kaposi's sarcoma cells: long-term culture with growth factor from retrovirus- infected CD4+) T cells. Science. 1988; 242:426-30.
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