Department of Hematology, SGPGIMS, Lucknow, India.
Int J Lab Hematol. 2021 Dec;43(6):1417-1423. doi: 10.1111/ijlh.13621. Epub 2021 Jun 11.
To assess the prevalence of early T precursor-acute lymphoblastic leukaemia (ETP-ALL), study its clinicopathological features and devise a 'flow score' based on immunophenotypic profiles.
This was a retrospective study where clinical and laboratory data of all consecutive T-ALL cases were analysed to identify features differentiating ETP from non-ETP-ALL. The utility of a flow score based on the five commonly used markers in leukaemia panels for T-ALL (CD34, CD8, CD5, CD13 and CD33) was evaluated to differentiate ETP from non-ETP-ALL.
Early T precursor-acute lymphoblastic leukaemia constituted 24.2% (n = 29) of all T-ALL cases. It was significantly more common in adults (30.2%) as compared to paediatric (17.5%) patients (P = .046). The median age of presentation was significantly higher than the non-ETP group. (24 vs 19 years; P = .01). Patients with ETP-ALL usually presented with organomegaly, lymphadenopathy, lower levels of haemoglobin, total leucocyte count, peripheral blood blast proportion and LDH levels as compared to non-ETP-ALL. The majority of ETP-ALL cases had L2 morphology with a moderate amount of cytoplasm showing frequent blebbing. A flow score cut-off value of ≥3 on ROC curve analysis had a sensitivity and specificity of 100% and 94.6% respectively.
Early T precursor-acute lymphoblastic leukaemia had unique clinical and laboratory features. The prevalence of this entity is more common in the adult population. A flow score based on a minimum of five widely used markers can confidently identify ETP-ALL and should be included in the primary panel of markers used for flow cytometric analysis.
评估早期 T 前体-急性淋巴细胞白血病(ETP-ALL)的患病率,研究其临床病理特征,并基于免疫表型特征制定“流式评分”。
这是一项回顾性研究,分析了所有连续 T-ALL 病例的临床和实验室数据,以确定区分 ETP 与非 ETP-ALL 的特征。评估基于白血病panel 中常用于 T-ALL 的五个常用标志物(CD34、CD8、CD5、CD13 和 CD33)的流式评分对区分 ETP 与非 ETP-ALL 的效用。
早期 T 前体-急性淋巴细胞白血病占所有 T-ALL 病例的 24.2%(n=29)。与儿科(17.5%)患者相比,成人(30.2%)中更为常见(P=0.046)。中位发病年龄明显高于非 ETP 组(24 岁与 19 岁;P=0.01)。与非 ETP-ALL 相比,ETP-ALL 患者通常表现为肝脾肿大、淋巴结病、血红蛋白、总白细胞计数、外周血原始细胞比例和 LDH 水平降低。大多数 ETP-ALL 病例具有 L2 形态,细胞质中等量,常出现泡状突起。ROC 曲线分析中,截值≥3 的流式评分具有 100%的敏感性和 94.6%的特异性。
早期 T 前体-急性淋巴细胞白血病具有独特的临床和实验室特征。这种实体在成人中更为常见。基于最少五个广泛使用的标志物的流式评分可以准确识别 ETP-ALL,应包含在流式细胞术分析中使用的初始标志物面板中。