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CD3+, CD56+ aggressive variant of large granular lymphocyte leukemia [see
comments]
TC Gentile, AH Uner, RE Hutchison, J Wright, J Ben-Ezra, EC Russell and TP Loughran
Department of Medicine, State University of New York at Syracuse.
Clonal expansions of CD3+ large granular lymphocytes (LGL) have been
classified as T-LGL leukemia. The majority of patients with T-LGL leukemia
have a chronic disease (years) manifested often by severe neutropenia,
rheumatoid arthritis, and mild-to-moderate splenomegaly. The characteristic
phenotype of the leukemic LGL is CD3+, CD8+, CD16+, CD57+, and CD56-. In
this report we describe an aggressive variant of T- LGL leukemia in which
leukemic LGL also expressed CD56, as identified by two-color flow-cytometry
analysis. In contrast to the chronic nature typical of T-LGL leukemia,
these patients presented with a severe systemic illness that was rapidly
progressive and resistant to treatment. Atypical clinical features included
rapidly increasing spleen size to massive proportions, extensive
lymphadenopathy, and the presence of B symptoms (fever, nightsweats, weight
loss). Hematologic and pathologic features were also unusual for T-LGL
leukemia. These patients had very high LGL counts at diagnosis (range
11,692 to 26,312 microL), which increased rapidly despite treatment.
Histopathologic examination of splenic sections showed extensive
infiltration of red pulp cords and sinuses by leukemic cells with atrophy
of the white pulp. These clinicopathologic features are similar to those
described for patients with natural killer cell (NK)-LGL leukemia, whose
cells are also CD56+. However, unlike NK-LGL leukemia, we could not show a
direct pathogenic role for Epstein-Barr virus (EBV), as Southern-blot
analyses using an EBV-joined termini probe were negative in these patients.
Our findings suggest that CD3+, CD56+ LGL leukemia is a distinct
clinicopathologic entity separate from the usual CD3+, CD56- T- LGL
leukemia. The expression on leukemic LGL of CD56, an adhesion molecule, may
determine the aggressive biologic nature of this newly described disease.
Volume 84,
Issue 7,
pp. 2315-2321,
10/01/1994
Copyright © 1994 by The American Society of Hematology
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