K. Matsue et al., POLYCLONAL B-CELL CHRONIC LYMPHOPROLIFERATIVE DISEASE WITH HAIRY-CELLMORPHOLOGY - A CASE-REPORT AND CLONAL STUDIES, American journal of hematology, 51(2), 1996, pp. 141-146
We describe a patient who has a chronic polyclonal B lymphocyte prolif
eration with a hairy-cell appearance, A 48-year-old Japanese woman wit
h marked splenomegaly, systemic lymphadenopathy, and leukocytosis was
referred to our hospital. Laboratory examination revealed marked polyc
lonal IgG hypergammaglobulinemia. Morphologic examination of the patie
nt's peripheral blood, including May-Giemsa staining and scanning elec
tron 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 analysi
s indicated the polyclonal nature of the proliferating B cells, This w
as 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 describ
ed previously, this case might be extremely rare, and has important im
plications for the pathogenesis of mature B cell lymphoproliferative d
iseases, including hairy cell leukemia. (C) 1996 Wiley-Liss, Inc.