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Heterogeneity of large granular lymphocyte proliferations: delineation of
two major subtypes
WC Chan, S Link, A Mawle, I Check, RK Brynes and EF Winton
Two major types of lymphocytosis of large granular lymphocytes (LGLs) were
observed. The proliferating LGLs in each type had distinct
immunophenotypes, functional characteristics, and probably belonged to
different cell lineages. The more common form (Type A) consisted of cells
derived from the T cell lineage and had the T suppressor/cytotoxic
phenotype (T11+, T3+, T8+). The expression of the Leu 7 and HLA-DR antigen
was variable. These cells did not have natural killer (NK) function but
showed a variable degree of antibody-dependent cell-mediated cytotoxic
(ADCC) activity. Neutropenia was invariably present and rheumatoid
arthritis and autoantibodies were frequent associations. These lymphocytes
had many similarities to the major type of LGLs present in normal adult
bone marrow. The other type of LGL lymphocytosis (Type B) consisted of
cells lacking the antigens T3 and T8 but expressing M1 and NKH1. These
cells possessed strong NK and ADCC activity but their cell lineage was not
clear. Neutropenia and autoimmune phenomena were not detected. The
cytochemical characteristics of the LGL granules from both types of
patients were similar but differences in ultrastructure were observed. LGLs
from Type B patients proliferated in the presence of Interleukin 2 (IL-2)
and 12- O-tetradecanoyl-phorbol-13 acetate (TPA). Significant changes in
their basic T11+, T3-, T8- phenotype were not observed. IL-2 and TPA,
however, had profound influence on the NK function of the cells with
enhancement in the case of IL-2 and marked suppression when stimulated by
TPA.
Volume 68,
Issue 5,
pp. 1142-1153,
11/01/1986
Copyright © 1986 by The American Society of Hematology
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