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T gamma-lymphoproliferative disease and related disorders in humans and
experimental animals: a review of the clinical, cellular, and functional
characteristics
CW Reynolds and KA Foon
T gamma lymphocytes are those lymphocytes that express receptors for both
the Fc portion of IgG and sheep erythrocytes. A very high proportion of
normal T gamma lymphocytes are large granular lymphocytes (LGL), the cell
responsible for most, if not all, natural killer (NK) and
antibody-dependent cell-mediated cytotoxicity (ADCC) in humans, rats, and
mice. In general, these cells are large lymphocytes with prominent
azurophilic granules in the cytoplasm. Recently, a group of
lymphoproliferative disorders made up predominantly of T gamma lymphocytes
has been described. The most common and best studied of these disorders we
refer to as "chronic T gamma-lymphoproliferative disease" (T gamma-LPD). In
most cases, this disease represents the abnormal expansion of LGL, which is
reflected by an increase in functionally active NK or ADCC effector cells.
The chronic T gamma-LPD lymphocytes are generally characterized as E- and
EA-rosette positive, acid-phosphatase, and beta-glucuronidase positive and
express the pan-T antigens OKT3/Leu-4, OKT11/Leu-5, the
suppressor-associated antigens OKT5,8/Leu-2, and the NK-associated antigens
Leu-7/HNK-1. Typically, the patients are older, predominantly males and
characteristically have a lymphocytosis of predominantly T gamma
lymphocytes with lymphocyte infiltration of the bone marrow and often the
spleen. While chronic T gamma-LPD is not usually an aggressive disease, the
patients are often neutropenic and have recurrent bacterial infections
requiring antibiotic therapy. Some patients have benefited from cytotoxic
chemotherapy., but most patients have not required chemotherapy. An
experimental LGL leukemia in F344 rats appears morphologically,
functionally, and clinically similar to the human chronic T gamma-LPD and
serves as an experimental model for further examining the ontogeny and
function of LGL and may be applicable for exploring new and more effective
means for the treatment of patients with chronic T gamma-LPD.
Volume 64,
Issue 6,
pp. 1146-1158,
12/01/1984
Copyright © 1984 by The American Society of Hematology
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