Poorva Vaidya , Huan-You Wang , Michelle D. Don , Brian R. Hinds , James K. Mangan
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引用次数: 0
摘要
近早期 T 细胞前体急性淋巴细胞白血病(ETP-ALL)是一种罕见的血液系统恶性肿瘤,二线治疗方案有限。T细胞白血病也很少伴发切缘白血病,而切缘白血病多见于髓源性白血病。我们介绍了一例被诊断为近似 ETP-ALL 的成年男性患者,其 IDH2 和 DNMT3A 基因突变提示其为髓细胞源性白血病,并伴有切端白血病。患者在接受高CVAD和奈拉滨治疗后病情恶化,我们用BCL-2抑制剂venetoclax和低甲基化药物地西他滨对其进行治疗。该治疗方案迅速诱导了骨髓反应,并缓解了切缘白血病。
IDH2-mutated near ETP-ALL with aggressive leukemia cutis and brisk response to venetoclax and decitabine
Near early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a rare hematologic malignancy, for which second line therapeutic options are limited. T-cell leukemias are also rarely associated with leukemia cutis, which is more often seen in leukemias of myeloid origin. We present the case of an adult male diagnosed with near ETP-ALL, with IDH2 and DNMT3A mutations, suggestive of a myeloid origin, and leukemia cutis. After the patient progressed on hyper-CVAD and nelarabine, we treated him with the BCL-2 inhibitor venetoclax and the hypomethylating agent decitabine. The regimen induced a rapid bone marrow response and resolution of the leukemia cutis.