原发性皮肤T细胞淋巴瘤

原发性皮肤T细胞淋巴瘤属结外非霍奇金淋巴瘤 ,以皮肤内辅助T细胞的单克隆扩增为特征。他与有相同组织学亚型的累及皮肤的原发性淋巴结淋巴瘤在临床及组织学特征、生物学行为及预后都明显不同。如今最常用的原发性皮肤T细胞淋巴瘤分类是EORTC分类 ,HTLV -Ⅰ可能与原发性皮肤T细胞淋巴瘤相关 ,有待进一步验证。原发性皮肤T细胞淋巴瘤有较为独特的免疫表型 ,T细胞受体基因重排检测有助于其早期诊断及疾病的转归和预防的判断     

第四讲 皮肤淋巴瘤皮肤T细胞淋巴瘤向大细胞淋巴瘤转化

阐述皮肤T细胞淋巴瘤向大细胞淋巴瘤转化的临床表现、组织病理、免疫组织化学染色、分子生物学特征、诊断和鉴别诊断与治疗。     

皮肤淋巴瘤 第四讲 皮肤T细胞淋巴瘤向大细胞淋巴瘤转化

阐述皮肤T细胞淋巴瘤向大细胞淋巴瘤转化的临床表现、组织病理、免疫组织化学染色、分子生物学特征、诊断和鉴别诊断与治疗。     

酷似皮下脂膜炎的T细胞淋巴瘤

酷似皮下脂膜炎的T细胞淋巴瘤是新近提出的一类周围T细胞淋巴瘤的亚型。本文就其临床表现、治疗与预后、组织病理、免疫表型与电镜检查和分子遗传学特征及其鉴别诊断等方面进行了讨论。     

鼻部NK/T细胞淋巴瘤累及皮肤

为了探讨鼻部NK／T细胞淋巴瘤累及皮肤的临床表现、病理、免疫表型及EB病毒相关性。通过临床表现及病程，分析原发及累及部位。通过UCHL－1，CD56,多克隆CD3，CD8，CD20，TIA－1，粒酶B（GrB）等对肿瘤细胞免疫表型进行研究，EBV－EBER探针原位杂交检测肿瘤细胞阳性率分析其病因及发病。结果本例皮肤及鼻部取材组织病理表现为坏死及中等大小的异型性肿瘤细胞增生浸润，UCHL－1＋，CD56＋，CD3＋，CD8－，CD20－，TIA－1＋，granzyme B ,为NK／T细胞淋巴瘤表型。EBV瘤细胞阳性率达60％，进一步证实为鼻部NK／T细胞淋巴瘤，EBV相关性。支持鼻部NK／T细胞淋巴瘤是具有特殊免疫表型，与EB病毒有高度相关性的独立疾病，临床进展迅速，病程短，提示为高度侵袭性。     

原发性皮肤T细胞淋巴瘤

原发性皮肤T细胞淋巴瘤属结外非霍奇金淋巴瘤，以皮肤内辅助T细胞的单克隆扩增为特征。他与有相同组织学亚型的累及皮肤的原发性淋巴结淋巴瘤在临床及组织学特征、生物学行为及预后都明显不同。如今最常用的原发性皮肤T细胞淋巴瘤分类是EORTC分类，HTLV-Ⅰ可能与原发性皮肤T细胞淋巴瘤相关，有待进一步验证。原发性皮肤T细胞淋巴瘤有较为独特的免疫表型，T细胞受体基因重排检测有助于其早期诊断及疾病的转归和预防的判断。     

皮肤的结外鼻型NK/T细胞淋巴瘤1例

对1例皮肤的结外鼻型NK/T细胞淋巴瘤临床病理结合免疫组化染色、EB病毒原位杂交及T细胞受体基因重排进行分析.右胫后多发结节,组织病理特征为肿瘤组织在真皮及皮下组织内弥漫性浸润,肿瘤组织具有血管中心性及血管破坏性特点,肿瘤细胞具有异型性.瘤细胞表达CD2,CD56,颗粒酶B,EBER阳性,未检测到TCR克隆性基因重排.诊断为皮肤的结外鼻型NK/T细胞淋巴瘤.皮肤的结外鼻型NK/T细胞淋巴瘤恶性度高、预后差;诊断有赖于组织病理及免疫表型检测及EBER原位杂交技术.     

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.     

皮肤血管内T细胞淋巴瘤:1例报道及文献复习

对1例血管内淋巴瘤进行免疫表型分析及原位杂交检测与 EB病毒的关系,并复习相关文献患者男,32岁,发热2个月,左大腿斑块1月.全身体检及辅助检查血小板90× 109,血沉24mm/h,C反应蛋白28.9 mg/L,LDH 448 U/L.病理学检查肿瘤细胞位于真皮层的小血管内,瘤细胞体积较大、核仁明显、胞浆嗜碱性,核分裂相可见.瘤细胞免疫表型CD3,CD45( LCA)呈阳性表达.原位杂交显示E2BERs阴性.患者经CHOP联合化疗一个疗程后获得缓解.血管内T细胞淋巴瘤是一种罕见淋巴瘤,此瘤的诊断必须依赖病理学及免疫组织化学检查,在不明原因发热伴发皮肤斑块时,应及时活检,积极联合化疗.     

第三讲原发性皮肤大细胞淋巴瘤的分类

原发性皮肤大细胞淋巴瘤（PCLC）可分为T细胞性和B细胞性两类。T细胞表型PCLCL[PCLCL(T)]可以CD30阳性与否预期其预后。CD30^＋PCLCL(T)和CD30^ 非皮肤原发性LCL（T）的预后差。从蕈样肉芽肿转化成的CD30^ 皮肤LCL的预后一般差，从淋巴瘤样丘疹病演变成的CD30^ -LCL仅系统性的预后差而皮肤CD30^ -LCL的预后则不差。原发性皮肤多形T细胞性淋巴瘤，大细胞型和原发性皮肤T－免疫母细胞性淋巴瘤的预后差。B细胞表型原发性皮肤大细胞淋巴瘤中绝大多数为原发性皮肤滤泡中心细胞性淋巴瘤，其预后较淋巴结滤泡中心细胞性淋巴瘤为好。     

Cutaneous T-cell lymphoma

The "Clinical Snapshot" series provides a concise examination of a clinical presentation including history, treatment, patient education, and nursing measures. Using the format here, you are invited to submit your "Clinical Snapshot" to Dermatology Nursing.     

Cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma (CTCL) is a neoplasm of helper T cells whose first manifestations usually appear in the skin. The various forms of CTCL are distinguished by both clinical features and histopathology. Early on, the diagnosis may be difficult to establish because of its numerous, and often non-specific, clinical presentations. Further, the pathological findings of early lesions may lack the diagnostic features observed in well-developed or advanced disease. The diagnosis of CTCL must be considered in any patient with a chronic, therapy-resistant condition of the skin. In patients with non-specific histological findings, a high index of suspicion and multiple biopsies may eventually lead to a diagnosis of CTCL. Once the diagnosis of CTCL is established, accurate staging is essential both for its effect on treatment decisions and for its prognostic value. In general, CTCL is a chronic, slowly progressive disease with a long evolution. The development of tumours is a poor prognostic sign, as is erythroderma. The Sezary syndrome is a distinct form of erythrodermic CTCL that is characterized by exfoliative erythroderma, lymphadenopathy, lymphocytosis, intense pruritus, and circulating large, abnormal lymphocytes (Sezary cells). When death does occur, it is most often due to septicemia. Treatment of CTCL must be tailored to the individual patient. The most commonly employed treatment options are photochemotherapy and topical chemotherapy.     

Subcutaneous T-cell lymphoma

A 70-year-old woman presents with a 2-year history of intermittent, subcutaneous nodules. The patient was otherwise asymptomatic. A biopsy specimen was consistent with a subcutaneous T-cell lymphoma, a rare subset of peripheral T-cell lymphoma; when accompanied by the hemocphagocytic syndrome, it can be rapidly fatal. The histopathologic characteristics and nature of the disease are discussed.     

Panniculitic T-cell lymphoma

Panniculitic T-cell lymphoma is a rare, aggressive variant of cutaneous T-cell lymphoma, with fewer than 100 cases described. The main problem is its diagnosis, as both the clinical and the histological features may simulate benign panniculitis. We present the case of a 34-year-old male patient, who had presented with an indurated plaque, sclerodermiform in appearance, on the front of the right thigh for 4 months, later accompanied by fever and constitutional symptoms. The initial diagnosis was cellulitis, but no clinical improvement was seen despite systemic antibiotic therapy. After two skin biopsies, the patient was diagnosed with panniculitic cutaneous T-cell lymphoma. The patient was treated with 8 cycles of CHOP chemotherapy, with resolution of the symptoms.     

Peripheral T-cell lymphoma

A 32-year-old man presented with a 5-year history of cutaneous nodules on his head and a diffuse, lichenified eruption. Histopathologic examination showed an atypical lymphocytic infiltrate. Immunophenotyping studies determined that the lymphocyte population to be CD4-positive, with partial loss of CD3 and CD7, and immunogenotyping studies showed a clonal rearrangement of the T-cell receptor. A positron-emission tomography scan showed increased uptake in cervical, axillary, and inguinal lymph nodes. A diagnosis of peripheral T-cell lymphoma was made, and the patient is undergoing chemotherapy.     

Cutaneous cytotoxic T-cell lymphoma

Cutaneous cytotoxic T-cell lymphoma is a rare variant of cutaneous T-cell lymphoma, which shows immunoreactivity for CD56+. We report on an 83-year old woman with numerous skin infiltrates and nodules mainly on her face and trunk. Histopathologic criteria lead to the diagnosis of cutaneous cytotoxic T-cell lymphoma. Bexarotene was not tolerated because of cardiac insufficiency, but treatment with methotrexate (7.5 mg once weekly) led to prompt regression of all lesions.     

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.     

Anaplastic large-cell T-cell lymphoma

A 64-year-old woman presented with 2 years of pruritic and ulcerated nodules and tumors on the trunk and arms. Histopathologic examination showed a diffuse infiltrate that consisted of predominantly small lymphocytes and scattered large atypical multinucleated cells positive for CD30. These findings were consistent with a diagnosis of anaplastic large-cell T-cell lymphoma, which is a CD30+ cutaneous lymphoma. This case highlights the importance of considering both histopathologic and clinical criteria in diagnosing a patient with a CD30+ cutaneous lymphoma.