医学
淋巴瘤
化疗
恶性肿瘤
移植
肿瘤科
造血干细胞移植
干细胞
内科学
遗传学
生物
作者
Dominique Bron,Laurence De Leval,Sandra Michiels,Sebastian Wittnebel
出处
期刊:Current Opinion in Oncology
[Ovid Technologies (Wolters Kluwer)]
日期:2021-09-01
卷期号:33 (5): 406-411
标识
DOI:10.1097/cco.0000000000000775
摘要
Hepatosplenic lymphoma (HSTCL) is a rare T-cell malignancy occurring in young males, associated with immune deficiency in 20% of the cases which, despite aggressive treatments, has a poor survival. Specific recommendations for first-line treatment remain debatable.Published data covering case reports or series of HSTCL concur that allogeneic stem cell transplant should be proposed as a consolidation after response to chemotherapy in all patients eligible for transplant. In the light of two recent clinical examples, we also confirm that specific chemotherapy and a first-line consolidation with allogeneic transplantation when a donor is available to represent a treatment of choice these rare and distinctive lymphomas. Recent molecular studies are summarized in this review and suggest potential targets for new therapeutic strategies.Major progresses have been achieved in improving the outcome of HSTCL l patients using intensive chemotherapy and allogeneic transplantation.
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