Winberg C D, Sheibani K, Krance R, Rappaport H
Blood. 1985 Oct;66(4):980-9.
Peripheral T cell lymphomas (PTCLs) form a morphologically heterogeneous group of non-Hodgkin's lymphomas that are generally considered to have immunophenotypes associated with mature T cells, usually those of helper T cells. We now describe and correlate the clinical, morphological, immunologic, and cell-kinetic findings based on the evaluation of eight tissue samples obtained at various times from a 13-year-old girl with PTCL. The early morphological expressions of this patient's PTCL were those of diffuse mixed-cell lymphoma and focal large-cell lymphoma (LCL) evolving from the histologic picture of an atypical immune response (AIR). These morphological findings were associated with an immature T cell immunophenotype associated with cortical thymocytes--namely, sheep erythrocyte rosette (sER)+, T11+, Leu-2a+, Leu-3a+, HLA-DR+, OKT6-, OKT9+, OKT10+--and with cell-kinetic findings that showed no evidence of aneuploidy and few cells in S phase. Diffuse pleomorphic LCL developed, which was associated with further dedifferentiation of the neoplastic T cells to the immunophenotype sER-, T11+, Leu-2a-, Leu-3a-, HLA-DR+, OKT6-, OKT9+, OKT10- and with cell-kinetic findings that demonstrated a distinct aneuploid population and a dramatic increase in the percentage of cells in the S phase. The immunophenotype of the PTCL at the time of the patient's death was T11-, Leu-2a-, Leu-3a-, HLA-DR+, OKT6-, OKT9+, OKT10-, an immunophenotype indistinguishable from that of a non-B non-T cell lymphoma. The immunologic findings in this case also suggest that an AIR in some cases may represent a prelymphomatous state or may be a morphological expression of PTCL. These observations indicate that PTCLs may be characterized by rapidly changing clinical, morphological, immunologic, and cell kinetic findings which are best evaluated by multidisciplinary studies.
外周T细胞淋巴瘤(PTCLs)是一组形态学上异质性的非霍奇金淋巴瘤,通常被认为具有与成熟T细胞相关的免疫表型,通常是辅助性T细胞的免疫表型。我们现在描述并关联基于对一名13岁PTCL女孩在不同时间获取的8个组织样本进行评估得出的临床、形态学、免疫学和细胞动力学结果。该患者PTCL的早期形态学表现为弥漫性混合细胞淋巴瘤和局灶性大细胞淋巴瘤(LCL),由非典型免疫反应(AIR)的组织学图像演变而来。这些形态学发现与一种与皮质胸腺细胞相关的未成熟T细胞免疫表型有关,即绵羊红细胞玫瑰花结(sER)+、T11+、Leu - 2a+、Leu - 3a+、HLA - DR+、OKT6 -、OKT9+、OKT10+,并且细胞动力学结果显示无非整倍体证据且S期细胞很少。弥漫性多形性LCL出现,这与肿瘤性T细胞进一步去分化为免疫表型sER -、T11+、Leu - 2a -、Leu - 3a -、HLA - DR+、OKT6 -、OKT9+、OKT10 -有关,并且细胞动力学结果显示有明显的非整倍体群体以及S期细胞百分比显著增加。患者死亡时PTCL的免疫表型为T11 -、Leu - 2a -、Leu - 3a -、HLA - DR+、OKT6 -、OKT9+、OKT10 -,这种免疫表型与非B非T细胞淋巴瘤难以区分。该病例的免疫学发现还表明,某些情况下的AIR可能代表淋巴瘤前期状态或可能是PTCL的一种形态学表现。这些观察结果表明,PTCLs可能具有临床、形态学、免疫学和细胞动力学结果快速变化的特征,而多学科研究最适合对其进行评估。