Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients.
Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients.
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肝脾 γδ T 细胞淋巴瘤是一种罕见的临床病理实体，预后较差：21 例患者的系列报告。
作者：
K. Belhadj;F. Reyes;J. Farcet;H. Tilly;C. Bastard;R. Angonin;E. Deconinck;F. Charlotte;V. Leblond;E. Labouyrie;P. Lederlin;J. Emile;B. Delmas;B. Arnulf;E. Zafrani;P. Gaulard
我们报告了21例肝脾γ δ T细胞淋巴瘤（HS γ δ TCL）患者的特征，这是自1994年以来在修订的欧美淋巴瘤（真实的）分类中被认可的一个实体。中位年龄为34岁。患者有脾肿大（n = 21）、肝肿大（n = 15）和血小板减少症（n = 20）。组织学检查结果是均匀的，并显示存在于脾红髓，肝脏和骨髓的血窦内的中型淋巴瘤细胞。骨髓受累通常是轻微的，但可以证明在所有患者的表型。细胞为CD 3 + CD 5-，表达γ δ T细胞受体，具有非活化细胞毒性细胞表型（TIA-1+，颗粒酶B-）。大多数患者为CD 4-/CD 8-（16/18）; CD 56+（15/18），表达Vdelta 1表位（Vd 1 +/Vd 2-/Vd 3-）（9/12）; EB病毒（EBV）阴性（18/20）。13例患者中有9例记录到等染色体臂7 q。8例患者既往接受过肾移植或有系统性狼疮、霍奇金病或疟疾病史。预后差，中位生存时间为16个月，除2例患者外，所有患者最终死亡，尽管巩固或挽救高剂量治疗。总之，HS γ δ TCL是一种具有独特临床、组织病理学和表型特征的疾病。联合表型的骨髓活检足以诊断，因此没有必要进行脾切除术。目前的治疗方式似乎对大多数患者无效。
We report on the characteristics of 21 patients with hepatosplenic gammadelta T-cell lymphoma (HSgammadeltaTCL), an entity recognized since 1994 in the Revised European American Lymphoma (REAL) classification. Median age was 34 years. Patients had splenomegaly (n = 21), hepatomegaly (n = 15), and thrombocytopenia (n = 20). Histopathologic findings were homogeneous and showed the presence of medium-sized lymphoma cells within the sinusoids of splenic red pulp, liver, and bone marrow. Marrow involvement was usually mild but could be demonstrated by phenotyping in all patients. Cells were CD3+CD5-, expressed the gammadelta T-cell receptor, and had a nonactivated cytotoxic cell phenotype (TIA-1+, granzyme B-). Most patients were CD4-/CD8- (16 of 18); CD56+ (15 of 18), expressed the Vdelta1epitope (Vd1+/Vd2-/Vd3-) (9 of 12); and were negative for Epstein-Barr virus (EBV) (18 of 20). Isochromosome arm 7q was documented in 9 of 13 patients. Eight patients had previously undergone kidney transplantation or had a history of systemic lupus, Hodgkin disease, or malaria. Prognosis was poor; median survival time was 16 months, and all but 2 patients ultimately died despite consolidative or salvage high-dose therapy. In conclusion, HSgammadeltaTCL is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Bone marrow biopsy with combined phenotyping is sufficient for diagnosis, and splenectomy is therefore unwarranted. Current treatment modalities appear to be ineffective in most patients.