Hepatosplenic and other gammadelta T-cell lymphomas.
Hepatosplenic and other gammadelta T-cell lymphomas.
复制标题
肝脾和其他 γδ T 细胞淋巴瘤。
DOI：
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发表时间：
2007
影响因子：
3.5
通讯作者：
P. Gaulard
中科院分区：
文献类型：
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作者：
F. Vega;L. Medeiros;P. Gaulard
2005年血液病理学学会/欧洲血液病理学协会研讨会第11次会议专门讨论肝脾T细胞淋巴瘤（HSTCL）。HSTCL是一种罕见的侵袭性淋巴瘤，以肝脾肿大、骨髓受累和外周血细胞减少为特征。HSTCL表现出独特的浸润模式;肿瘤细胞优先浸润脾红髓、肝和骨髓的窦状隙。肿瘤细胞具有非活化的细胞毒性T细胞免疫表型，并经常携带复发性细胞遗传学异常，等染色体7 q。大多数病例表达γ δ T细胞受体，但病例可能具有α表型，并被认为是疾病的变体。虽然HSTCL是表达γ δ T细胞受体的原型外周T细胞淋巴瘤，但γ δ T细胞的非HSTCL增殖可涉及其他淋巴结部位，主要是皮肤和粘膜。这些γ δ T细胞淋巴瘤在临床和组织学特征上表现出明显的异质性。与HSTCL相反，非HSTCL γ δ T细胞淋巴瘤通常具有活化的细胞毒性表型，并且很可能不是单一疾病实体。
The 2005 Society for Hematopathology/European Association for Haematopathology Workshop session 11 was dedicated to hepatosplenic T-cell lymphoma (HSTCL). HSTCL is a rare aggressive type of extranodal lymphoma characterized by hepatosplenomegaly, bone marrow involvement, and peripheral blood cytopenias. HSTCL exhibits a distinctive pattern of infiltration; tumor cells preferentially infiltrate the sinusoids of the splenic red pulp, liver, and bone marrow. The tumor cells have a nonactivated cytotoxic T-cell immunophenotype and frequently carry a recurrent cytogenetic abnormality, isochromosome 7q. Most cases express the gammadelta T-cell receptor, but cases can have an alphabeta phenotype and are considered to be a variant of the disease. Although HSTCL is the prototype peripheral T-cell lymphoma expressing the gammadelta T-cell receptor, non-HSTCL proliferations of gammadelta T cells can involve other extranodal sites, mainly skin and mucosa. These gammadelta T-cell lymphomas display marked heterogeneity in clinical and histologic features. In contrast with HSTCL, non-HSTCL gammadelta T-cell lymphomas frequently have an activated cytotoxic phenotype and most likely are not a single disease entity.