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Rearrangement of T-Cell Receptor Chain Gene as a Marker of Lineage and Clonality in T-Cell Lymphoproliferative Disorders¹

First Department of Internal Medicine [N. K., T. A., T. K., S. H., M. O.] and The First Department of Pathology [T. Y., K. O., M. K.], Fukuoka University School of Medicine, 7-45-1 Nanakuma, Jonan-ku, Fukuoka 814-01, Japan; The Department of Biophysics, Ontario Cancer Institute, University of Toronto, Ontario, M4X 1K9, Canada; [Y. T.] and the Department of Hematology, National Kyushu Cancer Center Hospital, 3-1-1 Notame, Fukuoka 815, Japan [M. K.]

We analyzed the rearrangement of T-cell receptor (TcR) chain gene in 88 cases of lymphoproliferative disorders; 31 acute lymphoblastic leukemias/lymphoblastic lymphomas (ALL/LBL); 27 adult T-cell leukemias/lymphomas, 9 angioimmunoblastic lymphoadenopathies (AILD); 10 T-cell lymphomas (non-Hodgkin's lymphoma); and 11 Hodgkin's disease. All of 9 T-ALL/LBL cases, of which 4 cases have neither ß nor gene rearrangement, had a new rearranged band of TcR locus. Ten of 16 B-lineage ALL/LBL had rearranged band(s) or deletion of TcR locus. The rearranged bands were recognized in 2 cases of AILD and 1 case of T-cell lymphoma. All cases of adult T-cell leukemias/lymphomas, 4 of AILD, 4 of T-cell lymphoma, and 8 of Hodgkin's disease had deleted TcR locus. Heterogeneous findings of TcR locus analysis were observed in AILD, T-cell lymphoma, and Hodgkin's disease. In 16 cases with TcR rearrangement, the J1 region was frequently used and the J2 region was rearranged in one AILD. It is suspected that J3 was used in one T-ALL/LBL. There was no correlation between the phenotypic pattern of CD3, CD4, CD8 in T-cell disorders and the rearrangement of the TcR gene. These findings suggest that the newly identified TcR chain gene rearranges at a very early stage of T-cell ontogeny; prior to the other TcR genes and perhaps at almost the same stage with CD7 expression. The TcR gene is useful in assessing clonality for the most immature T-cell neoplasms not showing rearrangement of the other TcR genes. This gene is not lineage specific; however, when used in conjunction with immunoglobulin heavy chain gene, it may be a useful tool to distinguish lymphoid lineage of ALL/LBL.

¹ This work was supported in part by Grant-in-Aid for Scientific Research 63570579 from the Ministry of Education, Science and Culture, Japan, and by Grant 1567 from the Naito Foundation and Grant 63235 from the Fukuoka Anti-Cancer Society.

² To whom requests for reprints should be addressed, at The First Department of Internal Medicine, Fukuoka University School of Medicine, 7-45-1 Nanakuma, Jonan-ku, Fukuoka 814-01, Japan.

Received 2/14/89. Revised 5/ 8/89. Accepted 5/11/89.