Clinicopathological features of lymphoma/leukemia patients carrying both BCL2 and MYC translocations

• Published in: Haematologica (Roma) Vol. 94; no. 7; pp. 935 - 943
• Main Authors: Tomita, Naoto, Tokunaka, Mami, Nakamura, Naoya, Takeuchi, Kengo, Koike, Junki, Motomura, Shigeki, Miyamoto, Ko, Kikuchi, Ako, Hyo, Rie, Yakushijin, Yoshihiro, Masaki, Yasufumi, Fujii, Soichiro, Hayashi, Takamasa, Ishigatsubo, Yoshiaki, Miura, Ikuo
• Format: Journal Article
• Language: English
• Published: Pavia Ferrata Storti Foundation 01.07.2009
• Subjects: Adult; Aged; Biological and medical sciences; Chromosome aberrations; Chromosomes - ultrastructure; Female; Genes, bcl-2; Genes, myc; Hematologic and hematopoietic diseases; Humans; Leukemia - diagnosis; Leukemia - genetics; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma - diagnosis; Lymphoma - genetics; Male; Medical genetics; Medical sciences; Middle Aged; Original; Prognosis; Time Factors; Translocation, Genetic; Treatment Outcome; Chromosomal aberration; Human; dual-hit lymphoma/leukemia; Hematology; Leukemia; Malignant hemopathy; Lymphoid neoplasm; Lymphoma; Lymphoproliferative syndrome; C-Onc gene; MYC; Cytogenetics; myc Gene; BCL2; Abnormal chromosome; Protooncogene; Cancer; Chromosome translocation
• ISSN: 0390-6078; 1592-8721
• DOI: 10.3324/haematol.2008.005355

1 Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Yokohama; 2 Department of Pathology, Tokai University School of Medicine, Kanagawa; 3 Department of Pathology, Cancer Institute, Japanese Foundation for Cancer Research, Tokyo; 4 Department of Pathology, St. Marianna University School of Medicine, Kawasaki; 5 Department of Chemotherapy, Kanagawa Cancer Center, Yokohama; 6 Division of Hemato-oncology, St. Marianna University School of Medicine, Yokohama City Seibu Hospital, Yokohama; 7 Department of Hematology, Tokai University School of Medicine, Kanagawa; 8 Department of Hematology, Yokohama City University Medical Center, Yokohama; 9 Cancer Center, Ehime University Graduate School of Medicine, Ehime; 10 Department of Hematology and Immunology, Kanazawa Medical University, Ishikawa; 11 Department of Internal Medicine, Okayama Red Cross General Hospital, Okayama; 12 Division of Hematology, Tenri Hospital, Nara and 13 Division of Hematology and Oncology, St. Marianna University School of Medicine, Kawasaki, Japan Correspondence: Naoto Tomita, M.D., Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Yokohama, Japan E-mail: cavalier{at}ch-yamate.dlenet.com Background: Lymphoid neoplasm with 18q21.3/ BCL2 and 8q24/ MYC translocation to immunoglobulin ( IG ) genes as dual-hit lymphoma/leukemia is very rare and known to have a poor clinical outcome. Design and Methods: To clarify the clinicopathological characteristics of this malignancy, we analyzed 27 cases of cytogenetically proven dual-hit lymphoma/leukemia. Results: Dual-hit lymphoma/leukemia was diagnosed at presentation in 22 cases and at relapse or disease progression in 5 cases. At the time of diagnosis of dual-hit lymphoma/leukemia, extranodal involvement was found in 25 cases (93%) and central nervous system involvement occurred in 15 cases (56%). The median survival and 1-year survival rate of the 27 cases were only 6 months and 22%, respectively, after diagnosis of the dual-hit lymphoma/leukemia. Seven cases of triple-hit lymphoma/leukemia (dual-hit lymphoma/leukemia with 3q27/ BCL6 translocation) were included; the median survival of these patients was only 4 months from the diagnosis of the dual-hit lymphoma/leukemia. The duration of survival of the patients with a triple-hit malignancy was shorter than that of the other 20 cases of dual-hit lymphoma/leukemia ( p =0.02). The translocation partner of MYC subdivided the dual-hit cases into two groups; 14 cases of IGH and 13 cases of IGK/L . The MIB-1 index was investigated in 14 cases with aggressive B-cell lymphoma, and was higher in the group with MYC-IGH translocation (n=7) than in the MYC-IGK/L group (n=7) ( p =0.02). Overall survival was not different between the MYC-IGH translocation group (n=14) and the MYC-IGK or MYC-IGL translocation group (n=13). Conclusions: Dual-hit lymphoma/leukemia is a rare but distinct mature B-cell neoplasm with an extremely poor prognosis characterized by frequent extranodal involvement and central nervous system progression with either of the translocation partners of MYC . Key words: BCL2 , MYC , dual-hit lymphoma/leukemia. Related Article Novel lymphoid neoplasms – the borderland between diffuse large B-cell lymphoma and Burkitt’s lymphoma Daphne de Jong Haematologica 2009 94: 894-896. [Full Text] [PDF]