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Natural killer-like T-cell lymphomas: aggressive lymphomas of T-large
granular lymphocytes [see comments]
WR Macon, ME Williams, JP Greer, RD Hammer, AD Glick, RD Collins and JB Cousar
Department of Pathology, Vanderbilt University Medical Center, Nashville,
TN, USA.
Natural killer (NK)-like T cells are major histocompatibility complex-
unrestricted cytotoxic T cells that are surface CD3-positive, express
NK-cell antigens, and rearrange their T-cell receptor. Most neoplasms
arising from this T-cell subpopulation have been a chronic
lymphoproliferative disease referred to as T-large granular lymphocyte
(LGL) leukemia. Only 10 NK-like T-cell lymphomas have been described in
detail previously; this study presents the clinicopathologic features of
six others and distinguishes these lymphomas from T-LGL leukemia. All
patients presented with B-symptoms and often had marked hepatosplenomegaly
without significant peripheral lymphadenopathy. Four of the six patients
were immunosuppressed. All had CD3, CD8, CD56- positive tumors, presumably
of hepatosplenic (n = 3), intestinal (n = 1), pulmonary (n = 1), or nodal
(n = 1) origin. Three patients had lymphomatous bone marrow infiltrates,
and four had peripheral blood involvement by neoplastic large lymphocytes,
some of which had a blastic appearance or resembled virocytes. Azurophilic
granules, ultrastructurally corresponding to cytoplasmic dense core and/or
double density granules, were seen in all cases. T-cell clonality was shown
in five tumors by Southern blot analysis, and three had abnormal
karyotypes. Two untreated patients died 20 days after presentation, and
three patients who received combination chemotherapy died within 5 months
of presentation. One patient remains in complete remission 22 months after
treatment. These findings suggest NK-like T-cell lymphomas are aggressive,
are clinicopathologically distinct from T-LGL leukemia, and should be in
the differential diagnosis of extranodal T-cell lymphoproliferations,
including those in immunosuppressed patients. Furthermore, the LGL
morphology, phenotype, and tissue distribution of some NK-like T-cell
lymphomas suggest they arise from thymic- independent T cells of the
hepatic sinusoids and intestinal mucosa.
Volume 87,
Issue 4,
pp. 1474-1483,
02/15/1996
Copyright © 1996 by The American Society of Hematology
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