Surface Marker Data and Functional Analysis of Hemophilia Patients with and without Antibodies to LAV1

Abstract

Patients with hemophilia have been reported to suffer from a series of immune dysfunctions in cellular and humoral immunity. In addition to “antigen overload” induced phenomena resulting from factor VIII and factor IX substitution, possibly via alloantigen “contamination” in the preparations, latent viral infections such as CMV, hepatitis B and non A/non B have been proposed as the responsible parameters (cf. [1] for review). The discovery of antibodies to LAV1/HTLV III in a group of these patients has sparked the discussion as to whether this type of virus contributes significantly to the pathogenesis of the immune system [2]. In contrast to the increasing percentage of hemophilia patients who have developed AIDS or AIDS-related symptoms from 1981/1982 to October 1984 [3], most recent studies indicate that the number of the cases of AIDS reported in hemophiliacs are not increasing as rapidly as those for other risk groups such as homosexuals, i. v. drug abusers, and their heterosexual partners [4–6]. Since the viral propagation in vivo is definitely related to the number of activated OKT4 positive T-cells [7], patients suffering from other multiple infections of viral, bacterial, or fungal origin are at highest risk to develop LAV1-induced immunodeficiency. This seems to be generally applicable to hemophiliacs as well. Furthermore, generalized lymphadenopathy has been reported in a number of hemophiliacs, however without direct evidence for LAV1 harboring in lymph node cells [8,9].