Rose Ashley, Zhang Ling, Jain Akriti G, Poovathukaran Babu Abida, Sokol Lubomir, Saeed Hayder, Mo Qianxing, Fan Wenyi, Zhang Xiaohui
Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA.
Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA.
Am J Hematol. 2023 Jun;98(6):913-921. doi: 10.1002/ajh.26918. Epub 2023 Apr 3.
T-cell prolymphocytic leukemia (T-PLL) is a rare, post-thymic T-cell neoplasm with a diverse clinical course. T-PLL is typically associated with a poor prognosis; however, a subset of patients have inactive disease on initial presentation. There is a lack of accurate delineation of the disease based on initial clinical presentation and pathological assessment, hindering clinical decision-making. To characterize and delineate disease subtypes based on initial clinical presentation and pathologic assessment, we retrospectively reviewed 81 patients with T-PLL treated at our institution. We compared patients with T-PLL who initially presented with a relatively indolent or stable disease course to those with an aggressive disease course. Clinicopathologic characteristics, overall survival (OS), and prognostic factors were analyzed. Patients with inactive disease had a significantly longer OS than patients with active disease. At diagnosis, presence of B symptoms, low hemoglobin, low platelet count, lymphocyte doubling time of fewer than 3 months, and abnormal cytogenetics were associated with shorter OS. Cell morphology, immunophenotype, absolute lymphocyte count, lactate dehydrogenase levels, involvement of liver, spleen, skin or central nervous system, presence of TCL1 rearrangement or inv (14)/t(14;14), presence of chromosome 8 abnormalities, and presence of deletion of 11q were not associated with significant OS difference among the patients. Receiving alemtuzumab as first-line treatment and consolidation with allogeneic hematopoietic stem cell transplant were associated with better outcomes. T-PLL inactive and active disease subtypes can exhibit overlapping yet different clinical and pathological features. We describe several prognostic factors at diagnosis that can be used for risk stratification and aid in guiding treatment decisions.
T细胞幼淋巴细胞白血病（T-PLL）是一种罕见的胸腺后T细胞肿瘤，临床病程多样。T-PLL通常预后较差；然而，一部分患者初诊时疾病处于静止期。基于初始临床表现和病理评估对该疾病缺乏准确的界定，这阻碍了临床决策。为了根据初始临床表现和病理评估来表征和界定疾病亚型，我们回顾性分析了在我院接受治疗的81例T-PLL患者。我们将初诊时表现为相对惰性或稳定病程的T-PLL患者与具有侵袭性病程的患者进行了比较。分析了临床病理特征、总生存期（OS）和预后因素。疾病静止期的患者OS明显长于疾病活动期的患者。诊断时，B症状、低血红蛋白、低血小板计数、淋巴细胞倍增时间少于3个月以及细胞遗传学异常与较短的OS相关。细胞形态、免疫表型、绝对淋巴细胞计数、乳酸脱氢酶水平、肝、脾、皮肤或中枢神经系统受累情况、TCL1重排或inv(14)/t(14;14)的存在、8号染色体异常的存在以及11q缺失的存在与患者之间的OS差异无显著相关性。接受阿仑单抗作为一线治疗并进行异基因造血干细胞移植巩固治疗与更好的预后相关。T-PLL静止期和活动期疾病亚型可表现出重叠但不同的临床和病理特征。我们描述了几种诊断时的预后因素，可用于风险分层并有助于指导治疗决策。
