Published online 16 June 2009
Haematologica, Vol 94, Issue 7, 935-943 doi:10.3324/haematol.2008.005355
Copyright © 2009 by Ferrata Storti Foundation
Naoto Tomita1 ,
Mami Tokunaka2 ,
Naoya Nakamura2 ,
Kengo Takeuchi3 ,
Junki Koike4 ,
Shigeki Motomura5 ,
Ko Miyamoto6 ,
Ako Kikuchi7 ,
Rie Hyo8 ,
Yoshihiro Yakushijin9 ,
Yasufumi Masaki10 ,
Soichiro Fujii11 ,
Takamasa Hayashi12 ,
Yoshiaki Ishigatsubo1 ,
Ikuo Miura13
Haematologica, Vol 94, Issue 7, 935-943 doi:10.3324/haematol.2008.005355
Copyright © 2009 by Ferrata Storti Foundation
Malignant Lymphomas |
Clinicopathological features of lymphoma/leukemia patients carrying both BCL2 and MYC translocations
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.
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