Kraus M D, Crawford D F, Kaleem Z, Shenoy S, MacArthur C A, Longtine J A
Department of Pathology, St. Louis Children's Hospital, Washington University School of Medicine, Missouri 63110, USA.
Cancer. 1998 Mar 1;82(5):983-92. doi: 10.1002/(sici)1097-0142(19980301)82:5<983::aid-cncr26>3.0.co;2-w.
An unusual case of a peripheral T-cell lymphoma of T gamma/delta hepatosplenic type (Tgamma/deltaHSL) that arose in a child 5 years after she received a heart transplant and 9 months after she developed Epstein-Barr virus (EBV) positive, B-cell lymphoid hyperplasia involving the tonsils is presented. The majority of the reported cases of Tgamma/deltaHSL have been described in young adult men without antecedent immunodeficiency; several well documented cases of Tgamma/deltaHSL in the posttransplant setting have been described previously, but none has been described in a child (or an adult) with a previously diagnosed EBV+ B-cell lymphoid hyperplasia.
Standard histologic, immunohistochemical, flow cytometric, and molecular genetic techniques were used in the evaluation of diagnostic material.
The patient's Tgamma/deltaHSL involved the spleen in a predominantly cordal pattern, and infiltrated the liver in an exclusively sinusoidal distribution. Bone marrow involvement was focal and interstitial. In all locations, malignant cells were of intermediate or large size and had oval nuclei with coarse chromatin, with a scant or moderate amount of eosinophilic cytoplasm. This Tgamma/deltaHSL expressed the characteristic CD2+, CD3+, [CD4- CD8-], Tdelta1+ phenotype, and malignant cells also expressed the natural killer cell marker CD56. Cytogenetic studies demonstrated isochromosome 7q with the addition of trisomy 8 as the tumor progressed. Southern blot analysis demonstrated clonal rearrangements of the gamma, delta, and beta loci of the T-cell receptor but did not identify EBV DNA within the tumor cells.
This case highlights the fact that a full range of lymphoid proliferations is possible in the posttransplantation period, and that a prior diagnosis of a B-cell disorder does not preclude the development of a subsequent T-cell posttransplant lymphoproliferative disorder (PTLD), which should be formally evaluated, especially if clinical circumstances appear atypical for a PTLD of the "usual" (EBV-related, B-cell) type.
本文报告了一例罕见的肝脾γ/δ型外周T细胞淋巴瘤（Tγ/δHSL），该患儿在接受心脏移植5年后、出现爱泼斯坦-巴尔病毒（EBV）阳性的扁桃体B细胞淋巴样增生9个月后发病。大多数已报道的Tγ/δHSL病例见于无免疫缺陷病史的年轻成年男性；先前已有几例记录良好的移植后Tγ/δHSL病例，但尚无儿童（或成人）先前诊断为EBV+B细胞淋巴样增生后发生该病的报道。
采用标准组织学、免疫组织化学、流式细胞术和分子遗传学技术评估诊断材料。
患者的Tγ/δHSL主要以条索状累及脾脏，并仅以窦状分布浸润肝脏。骨髓受累为局灶性和间质型。在所有部位，恶性细胞中等大小或较大，核呈椭圆形，染色质粗糙，嗜酸性细胞质稀少或中等。该Tγ/δHSL表达特征性的CD2+、CD3+、[CD4-CD8-]、Tδ1+表型，恶性细胞还表达自然杀伤细胞标志物CD56。细胞遗传学研究显示，随着肿瘤进展，出现7号染色体等臂体并伴有8号染色体三体。Southern印迹分析显示T细胞受体γ、δ和β基因座的克隆重排，但未在肿瘤细胞中鉴定出EBV DNA。
该病例突出了移植后时期可能出现各种淋巴样增殖这一事实，且先前诊断为B细胞疾病并不排除随后发生移植后T细胞淋巴增殖性疾病（PTLD），对此应进行正式评估，尤其是当临床情况对于“常见”（EBV相关的B细胞）型PTLD显得不典型时。
