Generalized lymphadenopathy and T cell abnormalities in hemophilia A

J Pediatr. 1983 Jul;103(1):18-22. doi: 10.1016/s0022-3476(83)80768-9.

Abstract

Two patients with hemophilia A had generalized lymphadenopathy, lymphopenia, elevated IgG values, depressed T4 (helper) lymphocytes, elevated T8 (suppressor) lymphocytes, and abnormally low T4/T8 ratios. One of the patients, who also had hepatosplenomegaly, underwent cervical lymph node biopsy; the node contained 43% T8-lymphocytes, a marked elevation over the small fraction of T8 cells usually found in lymph nodes. These patients may have a form of the acquired immune deficiency syndrome described in male homosexuals, Haitians, intravenous drug abusers, and recently, in patients with hemophilia. We studied T cell phenotypes in 43 patients with hemophilia. Fourteen of 28 patients given commercial factor VIII concentrates had abnormal T4/T8 ratios; none of nine patients who used cryoprecipitate had abnormal values. T4 helper cells were significantly lower, T8 suppressor cells significantly elevated, and T4/T8 ratios significantly lower in the lyophilized concentrate users and in patients with hemophilia as a total group. The type of therapeutic factor VIII replacement may alter the risk of developing T4/T8 abnormalities or AIDS.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Acquired Immunodeficiency Syndrome / transmission
  • Child
  • Factor VIII / adverse effects
  • Fibrinogen / adverse effects
  • Hemophilia A / complications
  • Hemophilia A / immunology*
  • Hemophilia A / therapy
  • Humans
  • Leukocyte Count
  • Lymphatic Diseases / etiology*
  • Male
  • T-Lymphocytes, Helper-Inducer / analysis*
  • T-Lymphocytes, Regulatory / analysis*

Substances

  • cryoprecipitate coagulum
  • Factor VIII
  • Fibrinogen