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Impaired natural killer activity and expression of interleukin‐2 receptor antigen in familial erythrophagocytic lymphohistiocytosis
Author(s) -
Kataoka Yoshiko,
Todo Shinjiro,
Morioka Yoshihito,
Sugie Katsuji,
Nakamura Yoshiaki,
Yodoi Junji,
Imashuku Shinsaku
Publication year - 1990
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19900501)65:9<1937::aid-cncr2820650911>3.0.co;2-w
Subject(s) - immunology , interleukin 2 , antigen , medicine , immune system , monoclonal antibody , stimulation , interleukin , receptor , cellular immunity , cytokine , antibody , endocrinology
A 1‐month‐old boy with familial erythrophagocytic lymphohistiocytosis (FEL) had a barely detectable natural killer (NK) activity of 0% to 7% (median, 0.5%) with an effector/target ratio of 20:1. The number of Leu7+ and Leu11+ cells was within normal range. In terms of interleukin‐2 (IL‐2) receptor antigens, IL‐2R/p55 (Tac) was marginally expressed whereas IL‐2R/p75‐related antigen recognized by YTA‐1 monoclonal antibody (MAb), i.e. , YTA‐1 antigen, was moderately expressed on the patient's mononuclear cells. Since the NK activity was restored in vitro by IL‐2 stimulation, insufficient in vivo IL‐2 production or altered cooperation of IL‐2R/p75 and IL‐2R/p55 (Tac) in the IL‐2 mediated immune response was suspected to be present. The induction of IL‐2R/p55 (Tac) in vitro was found to be impaired after stimulation with IL‐2, or YTA‐1 MAb. When the patient attained remission, the IL‐2R/p55 (Tac) induction had normalized, but low NK activity persisted. The results indicate that the IL‐2/IL‐2R system may play an important role in the etiology and pathogenesis of FEL.