Hemorrhagic sequelae of immune thrombocytopenic purpura in human immunodeficiency virus-infected hemophiliacs

Abstract

Clinical bleeding tendency and tests of immune function were studied prospectively in 11 human immunodeficiency virus (HIV)-infected hemophiliacs with immune thrombocytopenic purpura (ITP) and a platelet count less than 50,000/microL. These 11 patients represented 13% of a well-characterized cohort of 87 HIV + hemophiliacs. ITP developed a mean 3.5 years after seroconversion, mean platelet count at presentation was 36,000/microL (range 15,000 to 49,000/microL), and the mean age at seroconversion was 37.1 years. Nine patients (82%) suffered bleeding complications, including four with intracranial hemorrhage, which was fatal in three. At the onset of ITP, five had AIDS and six were asymptomatic. Mean T4 lymphocyte count at onset of ITP was 126 +/- 32/microL (range 5 to 267/microL). Sustained treatment responses occurred with intravenous gammaglobulin (2 of 2), one of whom spontaneously remitted, and with zidovudine (1 of 2), but not with steroids (0 of 6) or danazol (0 of 3). In conclusion, 13% of a cohort of HIV + hemophiliacs has developed ITP with platelets less than 50,000/microL, a significant proportion of whom (82%) have experienced bleeding complications. It is recommended that treatment for ITP in HIV + hemophiliacs be instituted once the platelet count falls below 50,000/microL in order to avoid serious hemorrhagic sequelae.

Role of splenectomy in the management of hemophilic patients with human immunodeficiency virus‐associated immunopathic thrombocytopenic purpura

“…In our experience, hemophiliacs with ITP face bleeding complications of increasing severity and frequency when platelet counts fall below 50,000-60,000/mm3 resulting in progressive ecchymoses and hemarthroses. Although intracranial hemorrhage is a rare occurrence in non-hemophilic ITP patients, a recent report showed that 4 of 11 seropositive hemophilic ITP patients suffered intracranial hemorrhage (2 with platelet counts above 30,000/mm3) [4].…”

“…ITP usually occurs as an isolated complication of HIV infection unrelated to CD4 lymphocyte counts and does not increase the risk for development of AIDS [ 1,3]. While ITP rarely results in bleeding complications in HIV-infected homosexuals or intravenous (IV) drug users, there may be a significant risk of bleeding when ITP complicates the course of a seropositive hemophiliac [4]. For hemophilia patients this adds yet another chronic problem during a period when they might otherwise be completely functional and asymptomatic from HIV infection.…”

Thrombocytopenia in HIV-infected and uninfected hemophiliacs

“…Furthermore, only platelet counts reported on study forms were included in this analysis, and data on platelet transfusions were not available. Therefore, cautious management of hemophiliacs with platelet counts of <50,000/mm 3 is still appropriate [12].…”

Low incidence of bleeding from HIV‐related thrombocytopenia in drug addicts and hemophiliacs: Implications for therapeutic strategies

“…Our data are in agreement with those of Goldsmith & Kisker (15), who reported successful conservative management of the HIVrelated thrombocytopenia in 7 adult hemophiliacs. However, a high frequency of bleeding and cases of severe intracranial hemorrhage has also been reported in hemophiliacs (16,17), particularly in those with factor VIII antibodies (18). In female drug addicts, there was a relatively high incidence of major bleeding from the uterus, so that more aggressive treatment should be considered.…”