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Blood, 1 January 2001, Vol. 97, No. 1, pp. 270-276
NEOPLASIA
BCL-6 protein is expressed in precursor T-cell lymphoblastic
lymphoma and in prenatal and postnatal thymus
Elizabeth Hyjek,
Amy Chadburn,
Yi Fang Liu,
Ethel Cesarman, and
Daniel M. Knowles
From the Department of Pathology, Weill Medical College
of Cornell University, New York, NY.
The organization and expression of the BCL-6 gene in normal and
neoplastic thymic T cells has not been fully determined. We examined 8 precursor T-cell lymphoblastic lymphomas (T-LBLs) and found significant
BCL-6 expression in 4 cases. Three of the BCL-6+ cases
expressed a common thymocyte phenotype (CD4+,
CD8+), and one expressed a precursor thymocyte phenotype
(CD4 , CD8). In 6 cases evaluated, including
those expressing BCL-6, molecular analyses demonstrated a germline
configuration of the BCL-6 gene and a wild-type BCL-6 gene first
exon-intron boundary region. We also evaluated 12 normal prenatal and
postnatal thymuses for BCL-6 protein. BCL-6 was expressed by most
cortical thymocytes and by scattered medullary thymocytes.
BCL-6+ cortical and medullary thymocytes also expressed
CD2, CD3, CD4, CD5, CD7, or CD8. We further analyzed the pattern of
BCL-2 and BCL-XL expression and their coexpression with
BCL-6 in normal thymus and T-LBL and compared it to that of follicle
centers of reactive lymph nodes and follicular lymphoma.
BCL-6+ cortical thymocytes coexpressed BCL-XL
but not BCL-2. All 4 BCL-6+ T-LBLs and 4 BCL-6 T-LBLs coexpressed BCL-2 and BCL-XL.
Conceivably, T-LBLs may arise through clonal expansion of cortical
thymocytes normally expressing the BCL-6 protein. The pattern of BCL-6,
BCL-2, and BCL-XL expression in cortical thymocytes is
highly reminiscent of germinal centers, and the abnormal coexpression
of BCL-2, BCL-XL, and BCL-6 in T-LBL is analogous to
coexpression in follicle center cell lymphomas, suggesting that
coexpression of these anti-apoptotic genes may contribute to the
pathogenesis of T-LBL.
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