期刊
CANCER GENETICS
卷 209, 期 6, 页码 267-271出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.cancergen.2016.05.070
关键词
Lineage switch; T-cell lymphoblastic lymphoma; acute monoblastic leukemia; t(6;11)(q27;q23); MLL-MLLT4 (AF6)
资金
- Japanese Ministry of Education, Culture, Sport, Science, and Technology (JSPS KAKENHI) [26461431]
- National Cancer Center Research and Development Fund [26-A-24]
- Japanese Ministry of Health, Labor and Welfare [23-004]
- MHLW KAKENHI [H26-Kakushintekigan-Ippan-133]
- Project for Development of Innovative Research on Cancer Therapeutics (P-Direct)
- Japan Leukaemia Research Fund
- Grants-in-Aid for Scientific Research [26461431] Funding Source: KAKEN
We present a patient with T-cell lymphoblastic lymphoma (T-LBL) harboring t(6;11)(q27;q23) that converted to acute monoblastic leukemia at relapse. A 27-year-old man developed T-LBL with a mediastinal mass. He exhibited several recurrences in the central nervous system and marrow. A fifth relapse occurred in the marrow, with 42.8% blasts with CD4, CD5, CD7, CD10, CD33, CD34, HLA-DR and cytoplasmic (cy) CD3. While achieving complete remission with nelarabine, sixth relapse occurred in the marrow with 6.8% blasts, which had characteristics of monoblastic features, 2 months later. Marrow blasts were positive for myeloperoxidase, CD4, CD33, CD56, CD64, and HLA-DR, but were negative for cyCD3, CD5, CD7, CD10, and CD34. Marrow cells at both the 5th lymphoid and 6th myeloid relapses had t(6;11)(q27;q23) and the same MLL-MLLT4 fusion transcript. In addition, the MLL-MLLT4 fusion sequences documented in the initial mediastinal cells were the same as seen in peripheral blood cells at the 6th relapse. The patient continues 7th remission after one course of gemtuzumab ozogamicin therapy followed by cord blood transplantation for more than 3 years. Sequential phenotypic and cytogenetic studies may yield valuable insights into the mechanism of leukemic recurrence and possible implications for treatment selection.
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