Pneumocystis carinii pneumonia among persons with hemophilia A.

This article describes 3 cases of Pneumocystis carinii pneumonia in heterosexual men with hemophilia A but without other underlying disease. None had a history of intravenous drug abuse. All 3 had lymphopenia and the 2 patients who were specifically tested had in vitro evidence of cellular immune deficiency. 2 patients have died and 1 remains critically ill. All 3 patients had received frequent injections of Factor VIII concentrate for many years but no 2 patients are known to have received concentrate from the same lots. The 1st patient a 62-year old resident of Westchester County New York with a history of chronic hepatitis presented with weight loss vague right upper quadrant abdominal discomfort cough and fever. The 2nd patient a 59-year old resident of Denver Colorado reported gradual weight loss dysphagia associated with pharyngitis aphthous-like ulcers and anterior cervical adenopathy. Laboratory evidence for cellular immune dysfunction included absent mitogen responses and depletion of the T-helper lymphocyte cell population relative increases in the T-suppressor cells and a resultant inverted T-helper/T-suppressor ratio. The 3rd patient a previously healthy 27-year old from Ohio developed fever urinary frequency splenomegaly anemia and lymphopenia. He showed the same evidence of cellular immune dysfunction as Patient 2. These represent the 1st reported cases of P. carinii pneumonia among hemophilia patients who have had no other underlying diseases and have not had therapy for immunosuppression. The clinical and immunologic features of these 3 cases are strikingly similar to those observed among homosexual men intravenous drug abusers and Haitian immigrants. Transmission of an agent through blood products is suspected to have occurred.