PEDIATRICS Vol. 85 No. 3 March 1990, pp. 323-330
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Natural Killer Cell Immunodeficiency in Siblings: Defective Killing in the Absence of Natural Killer Cytotoxic Factor Activity in Natural Killer and Lymphokine-Activated Killer Cytotoxicities

Atsushi Komiyama MD1, Hiroshi Kawai MD1, Akihiko Yabuhara MD1, Mitsuhiko Yanagisawa MD1, Yukiaki Miyagawa MD1, Masao Ota MD1, Hayato Hasekura MD1, and Taro Akabane MD1

1 From the Departments of Pediatrics and Legal Medicine, Shinshu University School of Medicine, Matsumoto, Japan

A immunodeficiency of natural killer cells as effectors for natural killer and lymphokine-activated killer cytotoxicities was first demonstrated in siblings. Two of three male siblings persistently lacked natural killer activity against K562 target cells as assayed by a 51Cr-release assay: percent lysis values were <1.0% as compared to the normal lymphocyte values of 43.5% ± 6.2% (mean ± SD). Their lymphocytes did not develop natural killer cell activity by changing effector to target ratios, prolonging the incubation time, or stimulating them with interferon-agr or interleukin 2. Numbers of lymphocytes bearing Leu-7, CD16, or NKH-1 were normal but those of Leu-7-, CD16+ cells were decreased as estimated by flow cytometry. Single cell-in-agarose assays showed normal numbers of natural killer cells capable of binding to a target cell but incapable of killing it. They had depressed levels of lymphokine-activated killer activity, which was totally eliminated by the treatment with OKT3 and complement. This result indicates that the patients' natural killer cells are also defective in the capacity to work as effectors for lymphokine-activated killer activity. The patients' natural killer cells did not produce natural killer cytotoxic factor activity. Antibody-dependent cellular cytotoxicity and cytotoxic T lymphocyte cytotoxicity were normal. These results demonstrate a selective natural killer cell deficiency as effectors for natural killer and lymphokine-activated killer cytotoxicities with a familial tendency, in which there is defective killing with the absence of natural killer cytotoxic factor activity.

Key Words: natural killer cell • lymphokine-activated killer cell • immunodeficiency

Submitted on February 27, 1989
Accepted on May 9, 1989