Chromosomal abnormalities define clonal proliferation in CD3‐ large granular lymphocyte leukemia

Abstract

Six patients with lymphoproliferative disorder of large granular lymphocytes (LDGL) were studied for chromosomal abnormalities. When the patients were divided into two groups depending on the expression of a mature antigen of T cell lineage, CD3, two with CD3+ phenotype had a stable disease, whereas three of the four patients with CD3- phenotype had marked hepatosplenomegaly and a highly aggressive disease. Chromosomal abnormalities were detected in four of six patients, indicating a clonal proliferation of large granular lymphocytes in individual patients. In particular, chromosomal abnormalities were found in three of the four patients with CD3- LDGL, all of which had a germ-line configuration of the T cell receptor beta-chain gene. Thus, the chromosomal abnormalities provide definitive evidence for the clonal origin of the expanded cells in the CD3- LDGL.

Aggressive natural killer-cell leukemia revisited: large granular lymphocyte leukemia of cytotoxic NK cells

“…[1][2][3][4][5] It was originally recognized in the mid-1980s as a non-T-cell type of aggressive large granular lymphocyte (LGL) leukemia. [6][7][8] Owing to its rarity and unique presentations, there have been many reports of small series of cases [9][10][11][12][13][14][15][16][17] or individual case reports. Patients present with fever, circulating leukemic LGLs with CD2 þ CD16 þ CD56 þ HLA-DR þ phenotype, anemia and thrombocytopenia.…”

ERK couples chronic survival of NK cells to constitutively activated Ras in lymphoproliferative disease of granular lymphocytes (LDGL)

“…Features of aggressive NK-LDGL include large numbers of circulating NK cells, hepatosplenomegaly, and a median survival time of only a few months. Most cases of aggressive NK-LDGL occur in the Far East and are the result of EBV infection (Ohno et al, 1988;Taniwaki et al, 1990). Patients with chronic NK-LDGL have lymphocytosis that is often associated with bone marrow failure consisting of moderate anemia and/or neutropenia (Tefferi et al, 1994).…”

Aggressive Granular Lymphocyte Leukemia of Natural Killer Cell Type in an Elderly Patient.

“…The possibility that the inv (9) (p23q3 1) in this patient was a constitutional abnormality was denied by the fact that the metaphases obtained by PHA stimulation showeda mosaic pattern, since constitutional abnormalities would have been seen in all metaphases. Moreover, it has been reported that NKcells can proliferate more rapidly than T cells when stimulated by 12) and that it is difficult to obtain metaphases without IL-2 in NKcell leukemia (13). In the present case, abnormal karyotypes were detected at a higher frequency by IL-2 stimulation than by PHAstimulation.…”