Tran Tuan, Krause John
Department of Pathology, Baylor University Medical CenterDallas Texas.
Proc (Bayl Univ Med Cent). 2018 Oct 22;31(4):511-513. doi: 10.1080/08998280.2018.1479579. eCollection 2018 Oct.
Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL) is a recently recognized subgroup of T lymphoblastic leukemia/lymphoma thought to be derived from lymphocytes at the ETP differentiation stage that have not irreversibly committed to the T-cell lineage. The definition of ETP-ALL is based on a unique immunophenotype that also expresses at least one myeloid or stem cell marker other than myeloperoxidase and monocytic markers. Correspondingly, ETP-ALLs are often found to express myeloid-associated mutations and have stem cell gene expression profiles. Because its morphology is nonspecific, recognizing the immunophenotype of this uncommon entity is important to separate it from other closely related acute leukemias. We report a case of ETP-ALL with and mutations and monosomy of chromosome 7 that did not initially respond to conventional ALL therapy but eventually responded to acute myeloid leukemia-type therapy, underscoring the interesting characteristics of this leukemia. Little information is available regarding monosomy 7 in this entity for the adult population.
早期T细胞前体急性淋巴细胞白血病/淋巴瘤（ETP-ALL）是T淋巴细胞白血病/淋巴瘤中一个最近才被认识的亚组，被认为起源于ETP分化阶段尚未不可逆地定向于T细胞谱系的淋巴细胞。ETP-ALL的定义基于一种独特的免疫表型，该表型除了髓过氧化物酶和单核细胞标志物外，还表达至少一种髓系或干细胞标志物。相应地，ETP-ALL常被发现表达髓系相关突变并具有干细胞基因表达谱。由于其形态不具有特异性，识别这种罕见实体的免疫表型对于将其与其他密切相关的急性白血病区分开来很重要。我们报告了一例伴有 和 突变以及7号染色体单体型的ETP-ALL病例，该病例最初对传统ALL治疗无反应，但最终对急性髓系白血病类型的治疗有反应，突出了这种白血病的有趣特征。关于成人人群中该实体7号染色体单体型的信息很少。
