The frequency, presentation, diagnosis and
clinical course of
Pneumocystis carinii infections (PCI) were studied during aerosolized
pentamidine prophylaxis (AP) and its impact on the spectrum of
AIDS-related and other pulmonary
infections in HIV-infected hemophiliacs. We conducted an open study on primary (PP) and secondary (SP) AP. Breakthrough P. carinii
infections (BPCI) and other infectious complications were analyzed retrospectively. Hemophiliacs without prior P. carinii
pneumonia (PCP) who had been reluctant to any prophylaxis and who developed PCP served as control group. Statistical analysis of the efficacy of prophylaxis was performed by calculating confidence intervals of binomial p. Of 73 hemophiliacs (56 on PP and 17 on SP) 10 developed BPCI (7 in PP and 3 in SP) during a mean observation time of 14.9 months (range 0.5-30); total 13.6% (6.7%; 23.7%), PP 12.5% (5.1%; 24%), SP 17.6% (3.7%; 43.4%), confidence intervals at a level of 95%. Three BPCI presented atypically with cavitation (1),
pneumothorax (2), Pneumocystis
pleuritis (1), dissemination (2) as compared to none in the control group. Sensitivity of bronchoalveolar lavage (BAL) was 88.9%, specificity 100% (both 100% in the control group). PCP was the leading
AIDS manifestation (21.3%), CNS manifestations taken together were more frequent (36.2%).
Bacterial pneumonia was the most frequent respiratory
infection. One patient of the study group with recurrent
pneumothorax possibly died of BPCI as compared to no BPCI-related deaths in the control group. Efficacy of prophylaxis in hemophiliacs was comparable to other risk groups. AP alone may be insufficient for the control of PCI in patients with long-term profound immunodeficiency, especially in SP. 30% of BPCI presented atypically.(ABSTRACT TRUNCATED AT 250 WORDS)