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儿童非霍奇金淋巴瘤的生物学特性与分期

Biology and staging of childhood non-Hodgkin lymphoma.

作者信息

Crist W M, Mahmoud H, Pickert C B, Pui C H

机构信息

St. Jude Children's Research Hospital, Department of Hematology and Oncology, Memphis, Tennessee 38105.

出版信息

An Esp Pediatr. 1988 Oct;29 Suppl 34:104-9.

PMID:3063151
Abstract

Childhood non-Hodgkin lymphomas are relatively common childhood cancers composed of malignant B lymphocytes or thymocytes at various stages of maturation, usually intermediate or late. Most often, they have a diffuse pattern of infiltration and are high grade, lymphoblastic, small noncleaved-cell (undifferentiated, Burkitt or non-Burkitt types) or large cell types. Primary site correlates with immunophenotype and histopathology. For example, most abdominal tumors are B cell and non-lymphoblastic types, and most thoracic primary tumors are of thymocytic origin. Skin presentations are rare, usually lymphoblastic in histology and often of the pre-B cell phenotype. An appropriate work-up and adequate staging is mandatory, since the selection of therapy depends heavily on disease stage. Histopathologic findings are additionally important in determining appropriate therapy for advanced-stage disease. Finally, cytogenetic studies of B and T cell malignancies have defined nonrandom abnormalities in many of these neoplasms and, together with molecular analyses, hold exciting promise for an improved understanding of tumor pathogenesis.

摘要

儿童非霍奇金淋巴瘤是相对常见的儿童癌症,由处于不同成熟阶段(通常为中期或晚期)的恶性B淋巴细胞或胸腺细胞组成。它们大多具有弥漫性浸润模式,且级别较高,为淋巴母细胞性、小无裂细胞(未分化、伯基特或非伯基特类型)或大细胞类型。原发部位与免疫表型和组织病理学相关。例如,大多数腹部肿瘤为B细胞和非淋巴母细胞类型,大多数胸部原发性肿瘤起源于胸腺细胞。皮肤表现罕见,组织学上通常为淋巴母细胞性,且往往为前B细胞表型。必须进行适当的检查和充分的分期,因为治疗方案的选择在很大程度上取决于疾病分期。组织病理学检查结果对于确定晚期疾病的适当治疗也很重要。最后,对B细胞和T细胞恶性肿瘤的细胞遗传学研究已经确定了许多此类肿瘤中的非随机异常,并且与分子分析一起,有望更深入地了解肿瘤发病机制。

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