Subcutaneous panniculitis-like T-cell lymphoma

This case report describes a 38 year-old lady with the clinical, histopathological, and immunohistochemical (IHC) changes of subcutaneous panniculitis-like T-cell lymphoma (SPTCL). The IHC findings revealed CD8 + and CD56 - cells, which are indicative of tumors which have an indolent course. Our patient is being managed with tapering doses of corticosteroids for the last nine months with good improvement.     

Subcutaneous panniculitis-like T-cell cutaneous lymphoma

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma classified in the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification as a unique extranodal lymphoma with characteristic by T cell receptor (TCR) gene rearrangement. We report here a case of SPTCL in a 22 year-old woman who had presented with variably sized multiple nodules on both her legs. Initial differential diagnoses considered were panniculitis and lupus panniculitis. The histopathology showed a predominantly subcutaneous lobular infiltrate with atypical lymphocytes, karyorrhexis and rimming of adipocytes by lymphoid cells. Immunohistochemistry showed CD4-, CD8+, CD56- T-cell phenotype. Although TCR rearrangement studies were not done, the above T-cell phenotype and sparing of epidermis and dermis suggested the possibility of an SPTCL alpha/beta type. The patient received five cycles of a cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) regimen which resulted in the regression in her skin lesions and constitutional symptoms.     

皮下脂膜炎样T细胞淋巴瘤1例

报告1例皮下脂膜炎样T细胞淋巴瘤。患者女,34岁。四肢出现疼痛性红斑、结节9年,伴有发热、消瘦等。组织病理学改变主要在皮下脂肪组织内,可见瘤细胞浸润于脂肪细胞间。免疫组化显示瘤细胞表达CD45Ro及CD68,不表达CD20。     

皮下脂膜炎样T细胞淋巴瘤1例

患者男性，14岁，因全身结节、斑块14个月，伴发热9个月收住院。7个月前有肝脾肿大、大网膜增厚，心包积液、胸腹水病史，皮质类固醇治疗有效，停药易反复。住院1周后，外周血3系迅速下降，肝功能迅速恶化，凝血障碍，并出现瘀点和上消化道出血。住院16天，自动出院，出院后6天死于上消化道出血。皮下结节处活检及免疫组化示：皮下脂膜炎样T细胞淋巴瘤。     

儿童皮下脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征一例附文献复习

患儿,女,8岁。皮肤结节、斑块8个月,右大腿肿胀1个月,伴发热1周。肝脏肋下约1~2 cm可触及、脾脏肋下约2~3 cm可触及,质软,无触痛。实验室检查:血细胞降低,肝酶、血脂、乳酸脱氢酶、C反应蛋白升高,血沉加快,凝血功能异常,铁蛋白升高。免疫组化:CD3、CD8、颗粒酶B、βF1阳性,Ki-67:约50%(+)。骨髓活检:骨髓增生活跃。皮损病理检查:皮下脂肪见异型细胞。诊断为脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征。我们对相关文献进行了复习。     

Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature

We present a remarkable case of subcutaneous panniculitic T-cell lymphoma (SPTL) that eluded diagnosis for 14 years and illustrates the importance of continued follow-up with repeat biopsy when SPTL is suspected. This case is unusual in that multiple biopsies demonstrated either a nonspecific panniculitis or lipomembranous panniculitis with calcified lipomembranes. A clinicopathologic review of 72 cases of SPTL from the English language literature is also presented, and approaches to diagnosis and treatment are reviewed.     

Subcutaneous panniculitis-like T-cell lymphoma with vacuolar interface dermatitis resembling lupus erythematosus panniculitis

Lupus erythematosus (LE) panniculitis (LEP) is a form of chronic cutaneous LE most often characterized by erythematous subcutaneous nodules. The histopathology of LEP may be distinctive, allowing a diagnosis even in the absence of any other LE features. Lymphocytic infiltration of the panniculus is termed lymphocytic lobular panniculitis. This entity has been reported in both LEP and subcutaneous panniculitis-like T-cell lymphoma. We describe a 67-year-old woman who presented with multiple dermal and subcutaneous nodules on her legs and hips. Biopsy specimens of roughly 15 lesions were interpreted as being diagnostic of LEP, however, with each subsequent biopsy specimen more atypical cells were observed. The patient responded only to moderate doses of oral prednisone, and failed intralesional triamcinolone acetonide, oral immunosuppressive agents, oral antimalarial agents, and oral thalidomide. Three years after the onset of her disease, ulcerated plaques and nodules developed. At this time a T-cell receptor gene rearrangement was present and subcutaneous panniculitis-like T-cell lymphoma was diagnosed. Despite multiple doses of chemotherapy she died approximately 1 year after diagnosis. Patients with apparent LEP that have atypical lymphocytes in their biopsy specimens should be followed up closely for the development of T-cell malignancy.     

皮下脂膜炎样T细胞淋巴瘤WHO-EORTC皮肤淋巴瘤分类的新概念

目的:研究皮下脂膜炎样T细胞淋巴瘤(SFTL)的临床病理特征、免疫表型、组织起源和预后.方法:按照2005年皮肤淋巴瘤世界卫生组织-欧洲癌症治疗研究组织(WHO-EORTC)新分类分析6例SPTL患者的临床资料.作常规组织病理检查和免疫组化标记,并对6例患者石蜡组织和1例患者冰冻新鲜组织切片进行15F1标记.结果:6例患者中男2例.女4例,中位年龄25.5岁.临床皮损以无痛性皮下结节和(或)斑块为主,少数患者有发热、贫血和脾大.瘤细胞主要限于皮下脂肪组织内,有异形性和核分裂,并见不同程度脂肪坏死和组织细胞吞噬现象.瘤细胞表达βF1、CD3、CD8、TIA-1、GB、LCA和CD45RO,不表达CD4、CD30、CD56、CD20和CD79a.6例SVTL患者平均随访37个月内均获缓解,仅1例在确诊42个月后因并发糖尿病、高血压及心衰而死亡.结论:SPTL是一种起源于αJβT淋巴细胞的罕见类型细胞毒性皮肤淋巴瘤,临床病程迁延反复,5年生存率高达80%以上.完整充分的免疫组化标记对SPTL的确诊和分型必不可少.     

Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological,immunophenotypic and molecular analysis of six patients

BACKGROUND: 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. OBJECTIVES: To describe the clinicopathological, immunophenotypic and molecular features of six U.K. patients with SPTCL. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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1例

报道1例皮下脂膜炎样T细胞淋巴瘤。患者女，34岁。全身反复出现肿块、结节性损害1年。4个月前部分肿块破溃，伴发热、乏力、贫血等症状。皮损组织病理显示皮下脂肪层的淋巴细胞、组织细胞浸润，脂膜炎样T细胞瘤。免疫组化证明下细胞来源，伴有TCRγ基因克隆性重排，诊断为脂膜炎样T细胞瘤。     

脂膜炎样T细胞淋巴瘤1例

报告1例皮下脂膜炎样T细胞淋巴瘤,患者女,44岁,左侧臀部出现红肿、结节,伴疼痛和发热半个月,皮损组织病理检查：皮下脂肪层有淋巴细胞、组织细胞浸润,免疫组化证明浸润淋巴细胞为T细胞来源,组织病理改变符合脂膜炎样T细胞淋巴瘤。     

皮下脂膜炎样T细胞淋巴瘤研究进展

皮下脂膜炎样T细胞淋巴瘤是指主要累及皮下脂肪细胞且与脂膜炎相似的一种原发于皮肤的外周T细胞淋巴瘤。本文对其临床特点、组织病理学特点、实验室检查、组织学来源,与EB病毒感染的关系、治疗及预后、诊断及鉴别诊断综述如下。