Abstract | BACKGROUND: 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.
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Authors | Naoto Tomita, Mami Tokunaka, Naoya Nakamura, Kengo Takeuchi, Junki Koike, Shigeki Motomura, Ko Miyamoto, Ako Kikuchi, Rie Hyo, Yoshihiro Yakushijin, Yasufumi Masaki, Soichiro Fujii, Takamasa Hayashi, Yoshiaki Ishigatsubo, Ikuo Miura |
Journal | Haematologica
(Haematologica)
Vol. 94
Issue 7
Pg. 935-43
(Jul 2009)
ISSN: 1592-8721 [Electronic] Italy |
PMID | 19535347
(Publication Type: Journal Article)
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Topics |
- Adult
- Aged
- Chromosomes
(ultrastructure)
- Female
- Genes, bcl-2
- Genes, myc
- Humans
- Leukemia
(diagnosis, genetics)
- Lymphoma
(diagnosis, genetics)
- Male
- Middle Aged
- Prognosis
- Time Factors
- Translocation, Genetic
- Treatment Outcome
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