Important note: Information in this article was accurate in 1985. The state of the art may have changed since the publication date.
Impaired immune regulation in children and adolescents with hemophilia and thalassemia in Israel.
Am J Pediatr Hematol Oncol. 1984 Winter;6(4):371-8. Unique Identifier : AIDSLINE MED/85196634 Umiel T; Friedman E; Luria D; Cohen IJ; Kaplinsky H; Netzer L; Pecht M; Trainin N; Zaizov R
Abstract:
The immune function was assessed in 22 children, adolescents and young adults with asymptomatic hemophilia, and 15 with thalassemia, in Israel. Five patients with hemophilia and two with thalassemia were found to be severely abnormal, having cutaneous anergy, very low T-helper cells, elevated T-suppressor cells, inverted T-helper/suppressor ratio, reduced response to mitogens and antigens, and nonfunctional NK cells. Four of the five hemophilia patients exhibited profound lymphopenia also. Decreased T-helper and mildly elevated T-suppressor cells with inverted T4/T8 ratio were observed in the hemophiliacs as a group. In the severe group, the reduction in T-helpers and T4/T8 ratio was more pronounced. The thalassemics as a group were found to have increased numbers of T-suppressor cells with decreased T-helper cells in those with intact spleen only. Both groups studied were found to have elevated IgG levels and low natural killer (NK) activity and normal response to mitogens. Cutaneous anergy was found to be a reliable indication for severe T-cell dysfunction and may serve as an early indication of impending AIDS. These results indicate that patients with hemophilia and with heavily hypertransfused thalassemia may be at increased risk of AIDS as they approach adolescence.
Keywords: Acquired Immunodeficiency Syndrome/*EPIDEMIOLOGY/IMMUNOLOGY Adolescence Adult Blood Transfusion/ADVERSE EFFECTS Child Child, Preschool Hemophilia/COMPLICATIONS/*IMMUNOLOGY Human Immunoglobulins/IMMUNOLOGY Infant Israel Killer Cells, Natural/IMMUNOLOGY Risk T-Lymphocytes, Helper-Inducer/IMMUNOLOGY T-Lymphocytes, Suppressor-Effector/IMMUNOLOGY Thalassemia/COMPLICATIONS/*IMMUNOLOGY JOURNAL ARTICLE
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Important note: Information in this article was accurate in 1985. The state of the art may have changed since the publication date.
