皮下脂膜炎样T细胞淋巴瘤1例

皮下脂膜炎样T细胞淋巴瘤临床上较少见,属于原发性皮肤淋巴瘤,病变主要累及皮下脂肪,呈脂膜炎样的原发性外周T细胞淋巴瘤.组织病理学改变为脂膜炎样异形淋巴细胞浸润,常伴有噬血细胞综合征,病情进展快,死亡率高.现将我科诊治的1例报告如下.     

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。     

皮下脂膜炎样T细胞淋巴瘤研究进展

皮下脂膜炎样T细胞淋巴瘤是近年新认识的少见的皮肤T细胞淋巴瘤,来源于αβTCRT细胞或γδTCRT细胞的细胞毒性T细胞。皮下脂膜炎样T细胞淋巴瘤主要表现为皮下多发性结节、斑块或皮下肿块,部分患者可伴有噬血细胞综合征,病情进展迅速。病理上可见皮下脂膜炎样改变,其中可见瘤细胞围绕单个脂肪细胞形成特征性的“花环样改变”,免疫组化分析为细胞毒性T细胞来源,表达αβTCR或γδTCR基因重排,对常规化疗反应欠佳,预后较差,其预后与其免疫表型有关。     

Epstein-Barr病毒感染及相关皮肤疾病

EB病毒是一种人类疱疹病毒,其原发感染多无临床症状或导致传染性单核细胞增多症。原发感染后,EB病毒终身潜伏于健康人体的B淋巴细胞。但是,部分感染者发展为慢性活动性EB病毒感染,或者出现一些EB病毒相关的恶性疾病,如Burkitt's淋巴瘤、鼻咽癌、霍奇金淋巴瘤、牛痘样水疱病样淋巴细胞增生性疾病、NK/T细胞淋巴瘤、皮下脂膜炎样T细胞淋巴瘤、血管免疫母细胞T细胞淋巴瘤。本文将详述EB病毒感染的特点、机制,并讨论EB病毒相关皮肤疾病的临床、病理特征。     

皮下脂膜炎样T细胞淋巴瘤1例及文献复习

患者男,73岁。左侧臀部及右足底部出现多发性红色皮下结节及斑块,伴发热。曾被误诊为"血管炎"及"结节性脂膜炎"。皮损组织病理示:肿瘤浸润皮下脂肪组织,真皮深层及皮下脂肪层小叶间隔及小叶内大量淋巴细胞及组织细胞浸润,呈团块状,见散在异型淋巴细胞。免疫组化示:CD3(+),CD45RO(+),CD30(+),CD8(+),CD20(-)和CD4(-)。患者于化疗半月后因继发严重肺部感染而死亡。诊断:皮下脂膜炎样T细胞淋巴瘤。本文对本病进行相关的文献复习。     

儿童皮下脂膜炎样T细胞淋巴瘤并发噬血细胞综合征一例

正皮下脂膜炎样T细胞淋巴瘤(subcutaneous panniculitis-like T-cell lymphoma,SPTCL)是一种原发于皮肤的外周T细胞淋巴瘤,主要累及皮下脂肪组织。本病临床少见,常伴噬血细胞综合征(hemophagocytic syndrome,HPS),预后差。笔者收治1例长期随访的皮下脂膜炎样T细胞淋巴瘤并先后两次在EB病毒感染活动期出现噬血细胞综合征的     

EB病毒感染相关性皮肤淋巴瘤

Epstein-BarrVirus（EBV）正常成年人群感染率达95%以上,其可以感染B细胞、T细胞、NK细胞以及上皮细胞,分为潜伏感染和增殖感染。EBV相关性皮肤病有NK/T细胞淋巴瘤,皮下脂膜炎样T细胞淋巴瘤,慢性活动性EB病毒感染,种痘样水疱病,种痘样皮肤T细胞淋巴瘤,蚊虫叮咬过敏症,传染性单核细胞增多症等,特别是鼻/鼻型结外NK/T细胞淋巴瘤、皮下脂膜炎样T细胞淋巴瘤和种痘样皮肤T细胞淋巴瘤较为严重,治疗效果不佳,死亡率高。     

皮下脂膜炎样T细胞淋巴瘤并嗜血细胞综合征和股静脉栓塞1例

皮下脂膜炎样T细胞淋巴瘤(subcutaneous panniculitis-like T-cell lymphoma, SPTCL)是一种少见的原发性皮肤T细胞淋巴瘤,临床和病理上有时被误诊为脂膜炎,如病程中出现嗜血细胞综合征(hemophagocytic syndrome,HPS),患者病情迅速恶化,死亡率高.现将我科诊治的1例SPTCL报道如下.     

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。     

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。     

Primary cutaneous CD8+ cytotoxic T‐cell lymphoma involving the epidermis and subcutis in a young child

CD8+ cytotoxic T‐cell lymphoma involving the skin represents a heterogeneous group of diseases that include subcutaneous panniculitis‐like T‐cell lymphoma, primary cutaneous aggressive epidermotropic CD8+ cytotoxic T‐cell lymphoma, and ‘type D’ lymphomatoid papulosis. In this report, we describe a case of CD8+ cytotoxic T‐cell lymphoma involving both the epidermis and subcutis. The patient was a 6‐year‐old girl who presented with a 3‐year history of multiple plaques on her trunk and legs. The lesions had relapsed twice but responded well to prednisone. Histopathologic examination showed the proliferation of atypical lymphocytes in the epidermis, dermis and subcutaneous tissue. On immunohistochemical analysis, the atypical lymphocytes were positive for βF1, CD3, CD8, perforin, granzyme B and TIA‐1, but negative for T‐cell receptor (TCR) γ, CD4, CD30 and CD56. It was difficult to classify this tumor in terms of the known types of cutaneous lymphoma, and this case should be differentiated with subcutaneous panniculitis‐like T‐cell lymphoma and primary cutaneous aggressive epidermotropic CD8+ T‐cell lymphoma.     

Subcutaneous Tissue Involvement of CD30-Positive Large Cell Lymphoma

There are several types of T-cell lymphoma presenting with subcutaneous tissue involvement showing different clinical features and prognoses. The entity of subcutaneous involvement of T-cell lymphoma should be differentiated. We report a case of CD30-positive large cell lymphoma which initially presented with subcutaneous tissue involvement only, but progressed to systemic dissemination.     

Systemic panniculitis‐like T‐cell lymphoma with involvement of mesenteric fat and subcutis

Subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) is an uncommon non‐Hodgkins primary cutaneous lymphoma that typically presents as subcutaneous nodules on the extremities or trunk. Here, we report an unusual case of systemic panniculitic T‐cell lymphoma with predominantly mesenteric extra‐cutaneous involvement and an aggressive clinical course with histopathologic and immunophenotypic features that mimic SPTCL. This case serves to expand the body of literature regarding systemic SPTCL‐like disorders with prominent extra‐cutaneous involvement.     

Composite cutaneous lymphoma of diffuse large B‐cell lymphoma‐leg type and subcutaneous panniculitis‐like T‐cell lymphoma

Composite lymphoma (CL) is a rare disease defined by the occurrence of two distinct lymphomas within a single tissue at the same time. We present the case of an 89‐year‐old male with a clinical history of immunoglobulin M monoclonal gammopathy of undetermined significance. The patient presented cutaneous eruption of nodules on the right bottom and arm. An excisional biopsy revealed cutaneous infiltration composed of two components. The first one consisted of large B‐cells with CD20+/MUM1+/BCL2+ phenotype whereas the second one involved the subcutaneous fat in a panniculitic manner, and was CD3+/CD8+/granzyme B+/TCRβF1+. The final diagnosis was CL of primary cutaneous large B‐cell lymphoma‐leg type (PCLBCL‐leg type) and subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL). We report and characterize for the first time coexistent PCLBCL‐leg type and SPTCL in a patient.     

Subcutaneous panniculitis‐like T cell lymphoma presented as erythema nodosum: A case report

Subcutaneous panniculitis‐like T cell lymphoma (SPTCL) is an extremely rare subtype of primary cutaneous T cell lymphomas mimicking panniculitis. Clinically, patients are usually presented with subcutaneous nodules, which usually leads to initial misdiagnosis as a benign cutaneous condition. Here, we report a 40‐year‐old female who presented with subcutaneous erythematous nodules on her extremities with fever. On the basis of the clinical presentations, histopathological features and immunohistochemical findings, a diagnosis of SPTCL was made. The patient was treated with the injection of recombinant human interferon α‐1b (30 μg) every other day for 3 months. The lesions gradually regressed. No new erythema nodules reappeared during the 10‐month follow‐up.     

Subcutaneous Panniculitis‐Like T‐Cell Lymphoma in Children: A Review of the Literature

Subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) is a type of skin lymphoma. Pleomorphic T‐cells infiltrate the subcutaneous tissue and mimic lobular panniculitis. We report a case occurring in a 12‐year‐old Chinese boy who presented with multiple indolent erythematous subcutaneous nodules on both extremities without systemic symptoms. He had a protracted course of multiple erythematous subcutaneous nodules for 1 year and underwent biopsy of lesional skin for histology and T‐cell receptor (TCR) gene analysis. Histopathology showed infiltration of medium to large atypical pleomorphic cells involving the subcutis with characteristic rimming of fat spaces. TCR gene rearrangement shows monoclonal rearrangements of the TCR β and γ chains. Immunophenotypic studies showed that CD3, CD4, and CD8 were strongly and diffusely positive in lesional cells and that CD56 was focally positive. In contrast, these cells were negative for CD20, CD30, and CD68. The combined morphology, characteristic histologic features, and positive T‐CR gene rearrangement supported a diagnosis of SPTCL. He is being treated with combination chemotherapy of cyclophosphamide, doxorubicin, vincristine, and prednisolone.     

皮下脂膜炎样T细胞淋巴瘤1例

患者女,19岁。左上臂外侧结节、溃疡4个月,泛发全身伴发热2月,加重20天。皮损组织病理示脂肪小叶及小叶间隔内见淋巴细胞弥漫性浸润性生长,肿瘤细胞体积较小,核深染,核仁可见,围绕单个脂肪细胞浸润形成"花环状"结构,并见细胞吞噬现象,脂肪组织坏死伴炎细胞浸润,肿瘤未侵犯真皮和表皮。免疫组化:CD3(+),CD8(-),CD45RO(+),CD20(-),CD56(+),Ki-67阳性细胞大于90%。诊断:皮下脂膜炎样T细胞淋巴瘤。入院3天后因多器官功能衰竭合并败血症而死亡。     

Epstein–Barr Virus‐Associated Hydroa Vacciniforme‐like Cutaneous Lymphoma in Seven Chinese Children

Abstract: Hydroa vacciniforme‐like cutaneous lymphoma is a rare type of Epstein–Barr virus‐associated lymphoma. We analyze clinicopathologic features of seven Chinese child patients with hydroa vacciniforme‐like cutaneous lymphoma and determine the pathogenic association of Epstein–Barr virus with this disorder. Clinical, histologic, and immunohistochemical features were reviewed. Skin lesions were subjected to Epstein–Barr virus‐encoded RNA detection by in situ hybridization. Serologic assay and quantitation of Epstein–Barr virus DNA were performed. All seven patients presented with facial vesicles, papulovesicles, and atrophic scarring. Histologically, skin specimens showed epidermal blister formation, and dense lymphoid infiltration throughout the dermis and subcutaneous tissue. The infiltrate was composed of both small and large irregular lymphocytic cells expressing CD45RO or CD56. Tumor cells positive for Epstein–Barr virus‐encoded RNA were detected in cutaneous infiltrates in all seven cases. Besides skin eruptions, all patients had systemic manifestations, such as intermittent fever, hepatosplenomegaly or lymph node enlargement. The amounts of Epstein–Barr virus DNA were increased in the peripheral blood in two detected cases, and antibody titers to Epstein–Barr virus revealed a chronic active Epstein–Barr virus infection in all cases. The diagnosis of hydroa vacciniforme‐like cutaneous lymphoma was made. Four of seven patients were treated with interferon α intramuscularly, and the skin eruptions improved. Hydroa vacciniforme‐like cutaneous lymphoma may present with severe facial edema, papulovesicular eruptions and permanent scars. The tumor cells often express natural killer‐ or T‐cell markers. The disease is often preceded by chronic active Epstein–Barr virus infection.     

Erythema multiforme‐like lesions in primary cutaneous aggressive cytotoxic epidermotropic CD8+ T‐cell lymphoma: A diagnostic and therapeutic challenge

Primary cutaneous aggressive cytotoxic epidermotropic CD8+ T‐cell lymphoma is an extremely rare, rapidly progressing, cutaneous lymphoma, with frequent systemic involvement and poor prognosis, that still represents a diagnostic and therapeutic challenge, especially in the early stage. Herein, we report a case of an elderly woman with a fulminant course, who at onset presented with clinical and pathological features mimicking erythema multiforme (EM) and treated with cyclosporine that led to rapid deterioration with fatal outcome 6 months after disease onset. Histopathology showed a lichenoid, epidermotropic and nodular, angiocentric, dermal and subcutaneous infiltrate of sF1, CD8+, CD45RA+ small to medium‐sized atypical lymphoid cells, which strongly expressed cytotoxic markers. Monoclonal T‐cell‐γ receptor was clonally rearranged and array‐CGH showed numerous chromosomal imbalances. This case evidences the clinical, pathological and therapeutic challenges involved in this tumor. The first biopsy showed an interface dermatitis‐like pattern, revealing the deceptive features that early cutaneous infiltrates of this aggressive lymphoma may have. A high suspicion for aggressive CTCL and a low threshold for repeat biopsies should be maintained when faced with rapidly progressing and/or ulcerative EM‐like lesions, especially if immunomodulatory therapy is being considered.     

Peripheral T-cell lymphoma with secondary epithelioid granulomatous cutaneous involvement: a clinicopathologic study of four cases

Epithelioid granuloma formation has rarely been observed in specific cutaneous lesions from T-cell lymphomas other than those of mycosis fungoides/Sézary syndrome (MF/SS). Three patients diagnosed with nodal and/or extranodal (tonsillar) non-Hodgkin's peripheral T-cell lymphoma (PTCL) and one patient with angioimmunoblastic T-cell lymphoma (AILD), developed specific cutaneous involvement showing prominent epithelioid cell and/or granulomatous inflammation. The original diagnostic lesions had no granulomatous features. In addition to a specific lymphomatous infiltrate, prominent dermal and/or subcutaneous granulomatous infiltrates were observed. Sarcoid-like granulomas were observed in two patients (one of them presented a granuloma annulare-like pattern in early lesions), granulomatous panniculitis was noted in one patient and in one patient with AILD, masses of epithelioid cells were noted. The clinicopathological features of cutaneous involvement by PTCL showing a florid epithelioid and/or granulomatous cell reaction are reviewed. Various histopathological patterns can be observed. The diagnostic difficulties of these cases are stressed.