Data from a cohort of 62 HIV-positive individuals with hemophilia or von Willebrands disease infected for a maximum period of 15 years were analyzed. The relation between CD4+ and total lymphocyte counts and their rate of decline was analyzed with respect to age at seroconversion, time of seroconversion, and development of disease and subsequent death. As expected, the CD4+ and total lymphocyte population decline correlated with increased probability of disease and death. The patients fell into two distinct categories with respect to this decline: those whose cell count declined steadily (single slope) and those whose cell count remained steady or decreased very slowly for a variable period and then declined sharply (double slope). Within this cohort, the presence of a double slope appears to indicate a poorer prognosis, as 9 of 18 of the patients who have died showed this pattern, whereas only 6 of 42 of the remaining patients have this pattern even though more than half of this group have CD4+ lymphocyte counts < 0.2 x 10(9)/L. In addition, the ratio of CD4+ lymphocyte count to total lymphocyte count decreased with increasing cumulative frequency of the cumulative incidence of disease and death and the overall probability of death in this cohort was lower than expected, being 30% 12 years after seroconversion.
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