儿童间变大细胞淋巴瘤预后相关危险因素研究进展

Prognostic factors of childhood anaplastic large cell lymphoma:an update

间变大细胞淋巴瘤(ALCL)为一种外周T细胞淋巴瘤,为儿童期常见的非霍奇金淋巴瘤类型之一。绝大多数儿童ALCL阳性表达CD30,并存在ALK基因重排及其相关癌性融合基因,即ALK+ALCL,为其共同性的免疫表型和细胞分子遗传学特征。ALCL在临床表现、组织病理学类型、细胞分子生物学、治疗反应和预后等方面存在较大异质性。不同亚型呈现出特定的免疫表型特征,基因变异谱和基因表达标签,多种信号转导通路。综合ALCL预后相关临床、肿瘤和治疗相关危险因素,有助于优化儿童ALCL危险度分层,指导临床个体化治疗,改善预后。文章对近年儿童ALCL预后相关因素方面的研究进展做一综述。

Anaplastic large cell lymphoma(ALCL),a pathologic entity of peripheral T-cell lymphomas,is one of the common non-Hodgkin's lymphomas in children.In the majority of childhood ALCL,they are characterized immunophenotypically by positive CD 30 staining,and cytogenetically by ALK gene rearrangements with resultant oncogenic ALK fusion proteins.Nevertheless,ALCL is highly heterogeneous in terms of clinical manifestations,cytogenetic and molecular aberrations,therapeutic responses,and clinical outcomes.Specific ALCL histologies might be associated with characteristic immunophenotypic features,mutation profiling and gene expression signature,involving distinct signal transduction pathways.Integration of clinical relevant,tumor-associated and therapy-related risk factors help to improve ALCL risk stratification,to direct risk-based individualized therapy and to further improve prognosis of childhood ALCL.In the present paper,recent advances in this particular research field are reviewed.