Impaired natural killer activity and expression of interleukin‐2 receptor antigen in familial erythrophagocytic lymphohistiocytosis

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 a...

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Bibliographic Details
Published in:Cancer 1990-05, Vol.65 (9), p.1937-1941
Main Authors: Kataoka, Yoshiko, Todo, Shinjiro, Morioka, Yoshihito, Sugie, Katsuji, Nakamura, Yoshiaki, Yodoi, Junji, Imashuku, Shinsaku
Format: Article
Language:English
Subjects:
Antibodies, Monoclonal - pharmacology
Biological and medical sciences
Cells, Cultured
Hematologic and hematopoietic diseases
Histiocytosis, Non-Langerhans-Cell - genetics
Histiocytosis, Non-Langerhans-Cell - immunology
Humans
Infant, Newborn
Interleukin-2 - pharmacology
Killer Cells, Natural - drug effects
Killer Cells, Natural - immunology
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Lymphocytes - drug effects
Lymphocytes - immunology
Male
Medical sciences
Monocytes - drug effects
Monocytes - immunology
Receptors, Interleukin-2 - analysis
Recombinant Proteins - pharmacology
Remission Induction
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Summary: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.
ISSN:0008-543X
1097-0142
DOI:10.1002/1097-0142(19900501)65:9<1937::AID-CNCR2820650911>3.0.CO;2-W