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Polyclonal B cell chronic lymphoproliferative disease with hairy cell morphology: A case report and clonal studies
Author(s) -
Matsue Kosei,
Nishi Haruko,
Onozawa Shigeru,
Itoh Mami,
Tsukuda Kohji,
Yamaguchi Masaki,
Nakao Shinji,
Kashimura Makoto
Publication year - 1996
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/(sici)1096-8652(199602)51:2<141::aid-ajh8>3.0.co;2-y
Subject(s) - biology , polyclonal antibodies , hairy cell leukemia , lymphoproliferative disorders , gene rearrangement , hairy cell , b cell , immunology , microbiology and biotechnology , pathology , antigen , antibody , lymphoma , leukemia , medicine , gene , genetics
We describe a patient who has a chronic polyclonal B lymphocyte proliferation with a hairy‐cell appearance. A 48‐year‐old Japanese woman with marked splenomegaly, systemic lymphadenopathy, and leukocytosis was referred to our hospital. Laboratory examination revealed marked polyclonal IgG hypergammaglobulinemia. Morphologic examination of the patient's peripheral blood, including May‐Glemsa staining and scanning electron microscopy, showed a monotonous proliferation of hairy‐appearing mature lymphocytes. An immunophenotypic study revealed an expansion of cells with mature B cell antigens positive for CD11c; however, light‐chain restriction was not seen. The lack of both immunoglobulin heavy‐chain and T cell receptor gene rearrangements by Southern blot analysis indicated the polyclonal nature of the proliferating B cells. This was confirmed further by a clonal analysis of the patient's lymphocytes using an X‐chromosome‐linked restriction fragment polymorphism within the X‐linked phosphoglycerate kinase (PGK) gene. Since chronic B cell lymphoproliferation with a hairy cell appearance has not been described previously, this case might be extremely rare, and has important implications for the pathogenesis of mature B cell lymphoproliferative diseases, including hairy cell leukemia. © 1996 Wiley‐Liss, Inc.