VH gene expression in hairy cell leukaemia

Authors: Maloum1; Magnac2; Azgui1; Cau3; Charlotte4; Binet1; Merle-Béral1; Dighiero2

Source: British Journal of Haematology, Volume 101, Number 1, April 1998 , pp. 171-178(8)

Publisher: Blackwell Publishing

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Abstract:

Hairy cells are characterized by their typical morphology and expression of specific surface antigens. Although their B-cell origin is now confirmed, their exact position in B-cell development remains unclear. To better define the origin of hairy cells, we analysed the immunophenotype and the Ig VH nucleotide sequence of seven cases of hairy cell leukaemia (HCL). Six of them were typical HCL and the remaining case corresponded to a variant HCL. Analysis of sequenced VH genes revealed that the VH1 family was used in one case, VH2 in one, VH3 in two, VH4 in two and VH5 in one. No preferential usage of VH genes was observed in this small series. In five cases high rates of somatic mutations were observed, with a predominance of mutations and replacements in CDR regions for three, indicating that these cells originate from cells that have been exposed to the hypermutation mechanism. The distribution of mutations in our small series provides some evidence of a selective mutational process.

Keywords: hairy cell leukaemia; VH genes; mutations; antigenic selection

Document Type: Original article

DOI: 10.1046/j.1365-2141.1998.00653.x

Affiliations: 1: Département d'Hématologie, CHU Pitié-Salpêtrière, 2: Service d'Immuno-Hématologie et d'Immunopathologie, Institut Pasteur, 3: Laboratoire d'Immunologie Cellulaire, CHU Pitié-Salpêtrière, 4: Laboratoire d'Anatomo-Pathologie, CHU Pitié-Salpêtrière, Paris, France

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