Department of Hematology, the First Affiliated Hospital, Harbin Medical University, Harbin 150001, China.
Department of Hematology, the First Affiliated Hospital, Harbin Medical University, Harbin 150001, China.
Transpl Immunol. 2024 Dec;87:102119. doi: 10.1016/j.trim.2024.102119. Epub 2024 Sep 2.
T-cell acute lymphoblastic leukemia (T-ALL) is a highly invasive hematological malignancy originated from T-lineage progenitor cells. The clonal proliferation and aggregation of primordial cells in bone marrow inhibit normal hematopoietic function, resulting in a series of hematocytopenia and infiltration symptoms. SET-NUP214 fusion is a recurrent event that is common in adult male T-ALL patients. It originates from chromosome del(9)(q34.11; q34.13) or t(9; 9)(q34; q34). Hematopoietic stem cell transplantation (HSCT) can significantly improve the survival rate of these patients. Due to the poor prognosis of patients and high relapse rate after remission, more effective strategies need to be proposed to improve prognosis and prevent relapse. Chidamide is a novel oral benzamide histone deacetylase inhibitor (HDACi) that can exert anti-tumor effects through multiple mechanisms. Here we report chidamide maintenance therapy after allo-HSCT in patients with SET-NUP214 fusion positive T-ALL. Both patients improved effectively during follow-up, confirming the efficacy of chidamide in improving the condition of these patients and may provide valuable clinical information for the treatment of this rare and understudied disease.
T 细胞急性淋巴细胞白血病（T-ALL）是一种起源于 T 系祖细胞的高度侵袭性血液系统恶性肿瘤。原始细胞在骨髓中的克隆性增殖和聚集抑制了正常造血功能，导致一系列血细胞减少和浸润症状。SET-NUP214 融合是一种常见于成年男性 T-ALL 患者的复发性事件。它源于染色体 del(9)(q34.11; q34.13)或 t(9; 9)(q34; q34)。造血干细胞移植（HSCT）可以显著提高这些患者的生存率。由于患者预后不良且缓解后复发率高，需要提出更有效的策略来改善预后和预防复发。西达本胺是一种新型口服苯甲酰胺组蛋白去乙酰化酶抑制剂（HDACi），可通过多种机制发挥抗肿瘤作用。我们在此报告 SET-NUP214 融合阳性 T-ALL 患者 allo-HSCT 后西达本胺维持治疗的情况。两名患者在随访期间均有效改善，证实了西达本胺改善患者病情的疗效，可能为治疗这种罕见且研究不足的疾病提供有价值的临床信息。
