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Clinical and morphologic features of B-cell small lymphocytic lymphoma with
del(6)(q21q23)
K Offit, DC Louie, NZ Parsa, D Filippa, M Gangi, R Siebert and RS Chaganti
Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York,
NY 10021.
Deletions of the long arm of chromosome 6 have been described in acute and
chronic lymphocytic leukemia (ALL and CLL) and prolymphocytic leukemia
(PLL), and have been associated with t(14;18)(q32;q21) in non- Hodgkin's
lymphoma (NHL). Of 55 cases of small lymphocytic (sm lym) NHL, deletions of
6(q21q23) were the most common recurring cytogenetic abnormality. Among 14
sm lym NHL with del(6)(q21q23), this abnormality occurred as a solitary
change in 3 cases. Each of these 3 cases, and 5 additional cases with
del(6q) and other abnormalities, showed atypical larger forms with the
morphologic appearance of prolymphocytes or paraimmunoblasts in the
peripheral blood. In comparison, of the 11 cases without del(6q) and
circulating abnormal cells, prolymphocytoid forms were observed in 4 cases
(P < .001). Of the 31 sm lym without del(6q), trisomies of chromosomes
3, 12, or 18, or t(11;14)(q13;q32) occurred in greater than 10% of cases.
Proliferation centers or infiltration by larger forms were observed in
similar proportions of tissue sections derived from sm lym NHL with or
without del(6q). The presence of the larger forms in the peripheral blood
did not have an adverse prognostic impact on the survival of the del(6q)
cohort, who experienced a median survival in excess of 6 years. All 14
cases of del(6q) sm lym NHL were characterized by a mature B-cell
phenotype. Expression of CD11c, a feature of a CLL/PLL variant previously
described, was not detected in 9 cases analyzed. In 5 cases of del(6q) sm
lym NHL, no circulating abnormal lymphocytes were noted. Twelve cases
presented with, or developed, clinical splenomegaly. These results suggest
that deletion of a gene or genes at 6q21-23 is associated with the
pathogenesis of a subset of B-cell sm lym NHL that may display larger
prolymphocytoid cells in the peripheral blood, but that follows a clinical
course typical of other well-differentiated lymphocytic neoplasms.
Volume 83,
Issue 9,
pp. 2611-2618,
05/01/1994
Copyright © 1994 by The American Society of Hematology
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