title: Development of lymphoproliferative disorder of granular lymphocytes in association with hairy cell leukemia.
abstract: Lymphoproliferative disorder of granular lymphocytes (LDGL) is a low grade T-cell disease characterized by clonal expansion of large granular lymphocytes of either T cell or natural killer (NK) cell lineage that express the cytotoxic T-cell/NK cell antigens CD16, CD56 and/or CD57. LDGL has been described in association with other malignancies, leading to theories of a common abnormal stem cell as well as development of the LDGL as an immune response to a primary tumor. We have studied 32 patients with hairy cell leukemia (HCL). In 15 patients (47%) we detected an increase in cells expressing cytotoxic T-cell/NK cell antigens. In 10(31%) patients these cells were of T cell lineage, while 5 patients (16%) had increased NK-cells. T cell clonality was detected by PCR in all cases with increased cytotoxic T-cells in which adequate DNA was obtained from peripheral blood. Since in 2 patients the LDGL was not present at diagnosis but developed during follow up, our data suggests that clonal LDGL may develop in response to the HCL. The significance of LDGL in the setting of HCL and flow cytometric evaluation of HCL versus LDGL will be discussed.
authors.affiliation: Laboratory of Pathology, Division of Clinical Sciences, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
authors.forename: X Y
authors.initials: XY
authors.lastname: Xie
authors.forename: L
authors.initials: L
authors.lastname: Sorbara
authors.forename: R J
authors.initials: RJ
authors.lastname: Kreitman
authors.forename: P I
authors.initials: PI
authors.lastname: Fukushima
authors.forename: D W
authors.initials: DW
authors.lastname: Kingma
authors.forename: M
authors.initials: M
authors.lastname: Stetler-Stevenson
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005434:Flow Cytometry; D015321:Gene Rearrangement; D006801:Humans; D016130:Immunophenotyping; D007943:Leukemia, Hairy Cell / Q000150:complications* / Q000276:immunology*; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D016176:T-Lymphocyte Subsets / Q000276:immunology*