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The lymphoproliferative disease of granular lymphocytes: updated criteria
for diagnosis
G Semenzato, R Zambello, G Starkebaum, K Oshimi and TP Loughran
Department of Clinical and Experimental Medicine, Padua University School
of Medicine, Italy.
The lymphoproliferative disease of granular lymphocytes (LDGL), also
referred to as LGL leukemia, is a heterogeneous disorder, but is
clinically, morphologically, and immunologically distinct. Although LDGL
has recently been included in the revised classification of lymphomas as an
independent clinical entity, no consensus exists on the criteria to
establish the diagnosis. The aim of this report was to refine the
parameters needed to make the diagnosis of LDGL. We studied 11 patients
with chronic granular lymphocytosis selected from among 195 cases observed
by our institutions from three different geographic areas (North America,
Europe, and Asia). These cases did not meet the current criteria for
inclusion in LDGL, since all patients had less than 2,000 GL/microL.
However, in each of these patients, we found evidence for expansion of a
discrete GL population. Clonal rearrangement of the T-cell receptor (TCR)
beta gene was found in peripheral blood mononuclear cells (PBMC) of all
nine patients with CD3+ LDGL. Using recently generated monoclonal
antibodies (MoAbs) against the TCR V beta gene regions, we identified a
unique TCR V beta on GL from each of three patients studied. In two
patients with CD3- LDGL, we also identified a restricted pattern of
reactivity, by staining with MoAbs against p58 antigen found on normal
natural killer (NK) cells. The clinical features of these 11 patients with
relatively low absolute number of GL were similar to those reported
previously for patients with greater than 2,000 GL/microL. These data
demonstrate that newer techniques such as MoAbs against V beta gene regions
and p58 molecules and molecular analyses are useful to identify expansions
of discrete GL proliferations. Demonstration of an expansion of a
restricted GL subset is evidence for the diagnosis of LDGL, even in
patients with a relatively low GL count. Our results also contribute to
distinguish between the end of normality and the beginning of pathology in
the broad spectrum of GL lymphocytoses.
Volume 89,
Issue 1,
pp. 256-260,
01/01/1997
Copyright © 1997 by The American Society of Hematology
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