Hoque S R, Child F J, Whittaker S J, Ferreira S, Orchard G, Jenner K, Spittle M, Russell-Jones R
Department of Dermatopathology, St John's Institute of Dermatology, St Thomas' Hospital, London, UK.
Br J Dermatol. 2003 Mar;148(3):516-25. doi: 10.1046/j.1365-2133.2003.05226.x.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma of the skin. In the World Health Organization classification of T-cell and natural killer cell lymphoma it is listed as an example of extranodal lymphoma. In practice, however, it is most likely to present to a dermatologist.
To describe the clinicopathological, immunophenotypic and molecular features of six U.K. patients with SPTCL.
The clinical, histological and immunophenotypic features were reviewed. T-cell receptor (TCR) gene analysis was performed on blood and tissue samples using polymerase chain reaction/single-strand conformational polymorphism analysis of the TCR-gamma gene using consensus primers. In situ hybridization was performed on lesional skin to detect mRNA for Epstein-Barr virus (EBV).
All patients presented with subcutaneous nodules, plaques or ulceration, and three had systemic symptoms. All biopsies exhibited an infiltrate of medium to large pleomorphic cells involving the subcutis with characteristic rimming of fat spaces. Five showed areas of necrosis, but only one showed marked cytophagia. In three cases the neoplastic cells did not express TCR-beta. One was strongly p53 positive, and the other two were CD56 positive. Both these patients showed epidermal involvement with lichenoid changes histologically, and both developed the haemophagocytic syndrome. The other three cases were TCR-beta positive, CD8 positive and CD56 negative. All cases were positive with pan T-cell markers and also for the cytotoxic granule protein T-cell intracellular antigen-1 and granzyme B. All cases were EBV negative both by immunostaining (latent membrane protein-1) and by in situ hybridization (EBV-encoded mRNA). TCR gene analysis revealed a T-cell clone in four of five cases; two of these patients had an identical T-cell clone in the peripheral blood. The median survival was 16 months. However, two of the three TCR-beta-negative patients have died, whereas none of the TCR-beta-positive patients has died.
This is the first series of SPTCL patients to be reported in the U.K. and the data support the view that there are two subsets of SPTCL: those derived from gammadelta T cells which carry a poor prognosis, and are usually CD56 positive, and a more indolent group derived from alphabeta T cells.
皮下脂膜炎样T细胞淋巴瘤（SPTCL）是一种罕见的皮肤细胞毒性T细胞淋巴瘤。在世界卫生组织的T细胞和自然杀伤细胞淋巴瘤分类中，它被列为结外淋巴瘤的一个例子。然而，在实际临床中，它最常被皮肤科医生诊断。
描述6例英国SPTCL患者的临床病理、免疫表型和分子特征。
回顾临床、组织学和免疫表型特征。使用聚合酶链反应/单链构象多态性分析TCR-γ基因，采用共识引物对血液和组织样本进行T细胞受体（TCR）基因分析。对病变皮肤进行原位杂交以检测爱泼斯坦-巴尔病毒（EBV）的mRNA。
所有患者均表现为皮下结节、斑块或溃疡，3例有全身症状。所有活检均显示中到大的多形性细胞浸润，累及皮下组织，脂肪间隙有特征性的边缘。5例有坏死区域，但只有1例有明显的噬血细胞现象。3例肿瘤细胞不表达TCR-β。1例p53强阳性，另外2例CD56阳性。这2例患者组织学上均有表皮累及伴苔藓样改变，且均发生噬血细胞综合征。另外3例TCR-β阳性、CD8阳性、CD56阴性。所有病例泛T细胞标志物以及细胞毒性颗粒蛋白T细胞内抗原-1和颗粒酶B均呈阳性。所有病例免疫染色（潜伏膜蛋白-1）和原位杂交（EBV编码的mRNA）均为EBV阴性。TCR基因分析显示5例中有4例存在T细胞克隆；其中2例患者外周血中有相同的T细胞克隆。中位生存期为16个月。然而，3例TCR-β阴性患者中有2例死亡，而TCR-β阳性患者均未死亡。
这是英国报道的首批SPTCL患者系列，数据支持以下观点：SPTCL有两个亚组，一组来源于γδT细胞，预后较差，通常CD56阳性；另一组来自αβT细胞，病情相对惰性。
