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T Machii, M Yamaguchi, R Inoue, Y Tokumine, H Kuratsune, H Nagai, S Fukuda, K Furuyama, O Yamada, Y Yahata and T Kitani
Department of Hematology and Oncology, Osaka University Medical School,
Japan.
Polyclonal B lymphocytosis was found in four patients having clinical and
hematologic features resembling those of hairy cell leukemia (HCL). All
four patients were women between 37 and 67 years of age. Three patients had
splenomegaly. Lymphadenopthy was absent or slight. Persistent lymphocytosis
was seen in all the patients, and anemia and/or thrombopenia was observed
in three of the patients. Abnormal lymphocytes have long microvilli and
prominent membranous ruffles on their surfaces. Bone marrow aspirates and
biopsy specimens showed increased numbers of abnormal lymphocytes with
round nuclei and abundant pale cytoplasm. Although these findings were
similar to those of HCL, studies of Ig gene rearrangements and expression
showed the polyclonal proliferation of B cells. We called this new disease
hairy B- cell lymphoproliferative disorder (HBLD). All four patients
exhibited a polyclonal increase in serum IgG. The morphology of the cells
in HBLD was more similar to that of leukemia cells of a variant form of HCL
(HCL-Japanese variant) than to typical HCL cells. The surface IgG+, CD5- ,
CD11c+, CD22+, CD24-, CD25- phenotype and the weak tartrate-resistant acid
phosphatase activity in the cells were identical to those of HCL cells of
the Japanese variant. Our findings suggest that the B cells in HBLD are the
nonmalignant counterpart of leukemic B cells in HCL- Japanese variant.
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