Krackeler Margaret Li, Broome Catherine, Lai Catherine
Georgetown University School of Medicine, Washington, DC, USA.
Medstar Georgetown University Hospital, Lombardi Cancer Center, Washington, DC, USA.
Stem Cell Investig. 2020 Dec 29;7:24. doi: 10.21037/sci-2020-035. eCollection 2020.
This is the first report of a complete remission in aggressive T-cell large granular lymphocytic (T-LGL) leukemia after treatment with pentostatin. The aggressive variant of the disease is rare, and traditional therapies include immunosuppressive agents, however, there is no standard consensus for treatment. Cytotoxic chemotherapy has led to remission in a few reported cases. We present this unique case as an alternative treatment for individuals refractory to chemotherapy. A 55-year-old African American male with hypertension, type II diabetes mellitus, hyperlipidemia, and gout presented with symptoms of multiple ecchymosis, fatigue, and weight loss. He was found to have splenomegaly (SM) and significant leukocytosis to 101 k/µL with 30% blasts on peripheral smear. Following bone marrow aspiration and biopsy with flow cytometry, he was diagnosed with aggressive T-LGL leukemia. The chemotherapy regimen hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) was initially chosen based on his clinical presentation but was refractory to treatment. His therapy was changed to alemtuzumab; however, patient tolerated poorly and did not respond. Pentostatin was added to alemtuzumab with improvement in clinical symptoms and laboratory parameters. The patient was transitioned to pentostatin monotherapy and achieved complete remission after 1 month. This report provides support for pentostatin as an effective treatment for patients with aggressive T-cell malignancies refractory to cytotoxic chemotherapy. Pentostatin has previously been studied to treat T-cell prolymphocytic leukemia (T-PLL), hairy cell leukemia, and marginal zone lymphoma. This case suggests an alternative, well-tolerated option that could be considered for initial therapy of aggressive T-LGL leukemia.
这是关于喷司他丁治疗侵袭性T细胞大颗粒淋巴细胞（T-LGL）白血病后完全缓解的首例报告。该疾病的侵袭性变体较为罕见，传统疗法包括免疫抑制剂，然而，对于治疗尚无标准共识。细胞毒性化疗在少数报道病例中导致缓解。我们呈现这一独特病例，作为对化疗难治个体的替代治疗方法。一名55岁患有高血压、II型糖尿病、高脂血症和痛风的非裔美国男性，出现多处瘀斑、疲劳和体重减轻症状。他被发现有脾肿大（SM），外周血涂片显示白细胞显著增多至101 k/µL，原始细胞占30%。经骨髓穿刺活检及流式细胞术检查后，他被诊断为侵袭性T-LGL白血病。最初根据其临床表现选择了hyper-CVAD（环磷酰胺、长春新碱、阿霉素和地塞米松）化疗方案，但治疗无效。他的治疗改为阿仑单抗；然而，患者耐受性差且无反应。在阿仑单抗基础上加用喷司他丁后，临床症状和实验室参数有所改善。患者转为喷司他丁单药治疗，1个月后实现完全缓解。本报告支持喷司他丁作为对细胞毒性化疗难治的侵袭性T细胞恶性肿瘤患者的有效治疗方法。喷司他丁此前已被研究用于治疗T细胞幼淋巴细胞白血病（T-PLL）、毛细胞白血病和边缘区淋巴瘤。该病例提示了一种可考虑用于侵袭性T-LGL白血病初始治疗的耐受性良好的替代选择。
