Abstract：
Background: T-prolymphocytic leukemia (T-PLL) is an aggressive hematologic malignancy. A portion of patients can be cured with alemtuzumab induction followed by allogeneic hematopoietic stem cell transplant, but patients who relapse after transplant have a poor prognosis, and there is no standard of care.Methods: We report a case of a 64-year-old man with relapsed JAK3-mutant T-PLL following allogeneic transplant who was treated with ruxolitinib and venetoclax.Results: Treatment with ruxolitinib and venetoclax resulted in a partial response including stabilization of the peripheral lymphocyte count, improvement in thrombocytopenia, decrease in splenomegaly, and a numerical reduction in the percentage of bone marrow involved by T-PLL. The combination was well tolerated with the exception of neutropenic infections.Conclusion: This case adds to the growing body of literature supporting venetoclax and rituximab as a viable treatment option for relapsed/refractory T-PLL with JAK-STAT alterations.
摘要：
背景：T淋巴细胞白血病（T-PLL）是一种侵袭性血液系统恶性肿瘤。部分患者可以通过阿仑单抗诱导，然后进行异基因造血干细胞移植来治愈。但是移植后复发的患者预后较差，没有标准的护理。方法：我们报告了一例64岁男性，在同种异体移植后复发JAK3突变T-PLL，接受了鲁索替尼和维奈托克治疗。结果：鲁索利替尼和维奈托克治疗导致部分反应，包括外周淋巴细胞计数稳定，血小板减少症的改善，脾肿大减少，以及T-PLL所涉及的骨髓百分比的数值降低。除中性粒细胞减少感染外，该组合的耐受性良好。结论：该病例增加了越来越多的支持维奈托克和利妥昔单抗作为JAK-STAT改变的复发/难治性T-PLL的可行治疗选择的文献。
