Impaired natural killer activity and expression of interleukin-2 receptor antigen in familial erythrophagocytic lymphohistiocytosis
✍ Scribed by Yoshiko Kataoka; Shinjiro Todo; Yoshihito Morioka; Katsuji Sugie; Yoshiaki Nakamura; Junji Yodoi; Shinsaku Imashuku
- Publisher
- John Wiley and Sons
- Year
- 1990
- Tongue
- English
- Weight
- 487 KB
- Volume
- 65
- Category
- Article
- ISSN
- 0008-543X
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✦ Synopsis
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 Leu'/+ and Leull+ cells was within normal range. In terms of interleukin-2 (IL-2) receptor antigens, IL-ZR/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. Cancer 65:1937-1941,1990. AMILIAL ERYTHROPHAGOCYTIC lymphohistiocytosis F (FEL), a rare and probably genetically transmitted disease affecting infants and very young children, is characterized by hepatosplenomegaly, pancytopenia, hypertriglyceridemia, hypofibrinogenemia, and meningeal involvement manifested by lymphohistiocytic pleocytosis associated with an increased protein level in the cerebral spinal fluid (CSF). The clinical course of the disease is usually acute and fatal, although a few short remissions are sometimes reported. In recent years, therapeutic results have improved with systemic administration of VP-1 6,s,6 central nervous system prophylaxis and bone marrow transplantation.' Although the direct etiology and the pathogenesis of this disease has not been determined, the clinical occurrence of frequently fatal infections suggests that immunodeficiency could be a component of FEL.