Fang Hong, Wang Sa A, You M James, Hu Shimin, Miranda Roberto N, Tang Zhenya, Lin Pei, Jorgensen Jeffrey L, Xu Jie, Thakral Beenu, Schlette Ellen J, El Hussein Siba, Bueso-Ramos Carlos, Medeiros L Jeffrey, Wang Wei
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Cytometry B Clin Cytom. 2022 Nov;102(6):440-447. doi: 10.1002/cyto.b.22095. Epub 2022 Sep 26.
The immunophenotype of pure erythroid leukemia (PEL) as determined by flow cytometry immunophenotypic analysis is not well characterized. The immunophenotypic difference between PEL and reactive conditions is under-explored.
We assessed and compared the immunophenotype of 24 PEL cases and 28 reactive cases containing early erythroid precursors by flow cytometry.
The neoplastic erythroid cells in all PEL cases were positive for CD36 and CD71. CD45 was also positive in all cases, but the expression level was often dimmer than granulocytes. CD117 expression ranged from partial to uniform, and CD235a was often only positive in the CD117-dim to negative cells, corresponding to more differentiated subset. PEL cases frequently (87%) showed decreased or negative CD38 expression, contrasting to reactive early erythroid precursors that showed bright CD38 (p < 0.0001). CD7 (25%) and CD13 (29%) aberrant expressions were only observed in PEL but not in the reactive erythroid cells. Normal early erythroid precursors in all reactive bone marrows showed partial expression of CD4; In contrast, aberrant CD4 expression was detected in 71% PEL cases, either uniformly positive (50%) or completely negative (21%). While normal/reactive bone marrows almost always contained a small subset of CD34-positive early erythroid precursors, the neoplastic pronormoblasts in all PEL cases were CD34 negative. Although not increased in number, CD34-positive myeloblasts were frequently detected in PEL and demonstrated an aberrant immunophenotype in 90% PEL cases.
PEL shows a distinctive immunophenotype which can be distinguished from reactive erythroid precursors by flow cytometry immunophenotyping.
通过流式细胞术免疫表型分析确定的纯红系白血病(PEL)的免疫表型特征尚不明确。PEL与反应性情况之间的免疫表型差异研究不足。
我们通过流式细胞术评估并比较了24例PEL病例和28例含有早期红系前体细胞的反应性病例的免疫表型。
所有PEL病例中的肿瘤性红系细胞CD36和CD71呈阳性。所有病例中CD45也呈阳性,但表达水平通常比粒细胞暗淡。CD117表达从部分阳性到均匀阳性不等,CD235a通常仅在CD117暗淡至阴性的细胞中呈阳性,对应于更分化的亚群。PEL病例经常(87%)表现出CD38表达降低或阴性,这与反应性早期红系前体细胞中CD38明亮表达形成对比(p < 0.0001)。CD7(25%)和CD13(29%)异常表达仅在PEL中观察到,而在反应性红系细胞中未观察到。所有反应性骨髓中的正常早期红系前体细胞均显示CD4部分表达;相比之下,71%的PEL病例检测到CD4异常表达,要么均匀阳性(50%),要么完全阴性(21%)。虽然正常/反应性骨髓几乎总是含有一小部分CD34阳性的早期红系前体细胞,但所有PEL病例中的肿瘤性原红细胞均为CD34阴性。虽然数量没有增加,但在PEL中经常检测到CD34阳性的原始粒细胞,并且在90%的PEL病例中表现出异常的免疫表型。
PEL表现出独特的免疫表型,可通过流式细胞术免疫表型分析与反应性红系前体细胞区分开来。