Gupta Richa, Garg Neha, Kotru Mrinalini, Kumar Dilip, Pathak Rajesh
Department of Pathology, University College of Medical Sciences Delhi, India.
Department of Paediatrics, Max Super Speciality Hospital Delhi, India.
Am J Blood Res. 2022 Feb 15;12(1):1-10. eCollection 2022.
T ALL may show variable morphological features and immunophenotypic analysis for characterisation of immature nature of these cells is needed to establish a diagnosis and distinguish from reactive conditions and mature T cell leukemias. Sometimes immaturity markers-CD34, TdT and HLA DR may not be expressed by blasts. The aim of the present study was to analyse immunophenotype of T ALLs especially with respect to absence of immaturity markers.
Thirty-eight cases of T ALL diagnosed over a period of two and half years were analysed retrospectively with respect to clinical features, haematological features and flow cytometric immunophenotyping for T, B, Myeloid and immaturity markers. Student's -test was used for comparing quantitative data and Chi-square test/Fishers exact -test for qualitative variables. value less than 0.05 was considered significant.
The most common T-lineage marker expressed was cCD3 and CD7 which were expressed in 100% cases followed by CD5 in 86.8% cases. The most common immaturity marker expressed was TdT (39.5% cases) followed by CD34 (34.2% cases). Thirteen cases (34.2%) were negative for all three of the immaturity markers i.e. TdT-/CD34-/HLADR. Absence of CD34 was associated with absence of expression of HLA DR (P<0.05) and aberrant expression of B lineage markers (P<0.05).
T-ALL is a rare and aggressive disease. Many cases lack immaturity markers viz, TdT, CD34 and HLADR. In such cases a comprehensive approach taking into account the clinical presentation, cytomorphology and immunophenotyping is diagnostic in experienced hands. Further, molecular studies may be needed to aid diagnosis.
T 淋巴母细胞白血病(T ALL)可能呈现多种形态学特征,需要进行免疫表型分析以确定这些细胞的不成熟性质,从而建立诊断并与反应性疾病和成熟 T 细胞白血病相鉴别。有时原始细胞可能不表达不成熟标志物——CD34、末端脱氧核苷酸转移酶(TdT)和人类白细胞抗原 DR(HLA DR)。本研究的目的是分析 T ALL 的免疫表型,尤其是关于不成熟标志物缺失的情况。
回顾性分析了两年半时间内诊断的 38 例 T ALL 病例的临床特征、血液学特征以及 T、B、髓系和不成熟标志物的流式细胞免疫表型。采用 Student's t 检验比较定量数据,采用卡方检验/Fisher 精确检验分析定性变量。P 值小于 0.05 被认为具有统计学意义。
最常表达的 T 系标志物是胞质 CD3(cCD3)和 CD7,100%的病例表达,其次是 CD5,86.8%的病例表达。最常表达的不成熟标志物是 TdT(39.5%的病例),其次是 CD34(34.2%的病例)。13 例(34.2%)病例的所有三种不成熟标志物即 TdT-/CD34-/HLA DR 均为阴性。CD34 的缺失与 HLA DR 的表达缺失相关(P<0.05)以及 B 系标志物的异常表达相关(P<0.05)。
T ALL 是一种罕见且侵袭性的疾病。许多病例缺乏不成熟标志物,即 TdT、CD34 和 HLA DR。在这种情况下,对于经验丰富的人员,综合考虑临床表现、细胞形态学和免疫表型的方法具有诊断价值。此外,可能需要分子研究来辅助诊断。