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Blood, 15 April 2001, Vol. 97, No. 8, pp. 2401-2405
NEOPLASIA
Analysis of BCL-6 mutations in classic Hodgkin disease
of the B- and T-cell type
Volkhard Seitz,
Michael Hummel,
Ioannis Anagnostopoulos, and
Harald Stein
From the Institute of Pathology, Consultation and
Reference Center for Lymph Node Pathology and Haematopathology,
University Hospital Benjamin Franklin, Free University Berlin, Germany.
BCL-6 is essential for germinal center formation and
thus for affinity maturation of immunoglobulin (Ig) genes
by somatic mutations. The 5'-noncoding region of the BCL-6
gene is even a target for the mutation machinery. Translocations of the
BCL-6 gene to heterologous promoters and mutations of its
5'-noncoding regulatory region were reported to be potential mechanisms
for deregulating BCL-6 expression and for playing a role in the genesis of non-Hodgkin lymphoma. In line with this hypothesis is the
observation that B-cell lymphoma with somatic mutations, such as
diffuse large B-cell lymphoma and follicular lymphoma, also carry
BCL-6 mutations, some of which are recurrently detectable.
Classic Hodgkin disease (cHD) is also derived from B cells with high
loads of somatic mutations and thus a further candidate for
BCL-6 mutations. To determine the presence and potential
role of BCL-6 mutations in cHD, the 5'-noncoding
BCL-6 proportion of single Hodgkin and Reed-Sternberg (HRS)
cells from 6 cases of cHD and 6 cases of HD-derived cell lines was
analyzed. All B-cell-derived HD cases and cell lines harbored
BCL-6 mutations. In contrast, both T-cell-derived HD cases
and cell lines were devoid of BCL-6 mutations. With only one exception, there were no lymphoma-specific recurrent
BCL-6 mutations detected, and BCL-6 protein was absent from
the HRS cells of most cases. In conclusion, (1) somatic
BCL-6 mutations are restricted to cHD cases of B-cell
origin, and (2) the BCL-6 mutations represent mostly
irrelevant somatic base substitutions without consequences for
BCL-6 protein expression and the pathogenesis of cHD.
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