A Case of Hemophilia B with Recurrent Haemophilus Influenzae Pneumonia and Abnormalities of Lymphocyte Functions

• Published in: The Japanese Journal of Pediatric Hematology Vol. 1; no. 1-2; pp. 154 - 159
• Main Authors: TANAKA, Yoshito, FUJITA, Atsushi, SAKANO, Takashi, UEDA, Kazuhiro, KOBAYASHI, Masao, KITTAKA, Eishi, TANIGUCHI, Hiroshi
• Format: Journal Article
• Language: Japanese
• Published: THE JAPANESE SOCIETY OF PEDIATRIC HEMATOLOGY/ONCOLOGY 30.05.1987
• Subjects: AIDS-related complex (ARC); Haemophilus influenzae pneumonia; hemophiliacs
• ISSN: 0913-8706; 1884-4723
• DOI: 10.11412/jjph1987.1.154

We treated an 11-year-old boy with hemophilia B who had recurrent pneumonia due to Haemophilus influenzae and who showed profound immunological abnormalities compatible with AIDS-related complex (ARC). He had been frequently treated with prothrombin complex concentrates since early childhood. At 11 years of age, he started to have recurrent pneumonia due to Haemophilus influenzae and abnormalities of lymphocyte functions. He was admitted to hospital six times over one year due3 to pneumonia with high fever and cough. At the time of these admissions, H. influenzae was always isolated from his sputum. Abnormal immunological findings were as follows : marked decrease of the number of OKT 4-positive cells, OKT 4/8 ratio, Tac-positive cells, and interleukin-2 production, absence of delayed-type hypersensitivity and diminished lymphocyte blastogenesis and immunoglobulin production as stimulated by pokeweed mitogen. Furthermore, a decrease of Leu 11-positive cells and depletion of natural killer cell activity were found. Serum human immunodeficiency virus antibody was positive by ELISA and Western Blot. A careful evaluation of clinical symptoms and immunological studies are necessary in hemophiliacs who receive long-term treatment with commercial coagulation factor concentrates.