Dermatologic radiotherapy of primary cutaneous follicle center cell lymphoma

A retrospective study was performed to evaluate the results of dermatologic radiotherapy in a large series of patients affected by primary cutaneous B-cell lymphomas. The study included 104 patients with cutaneous follicle center cell lymphoma treated with orthovoltage radiotherapy from 1973 to 2000. The total doses ranged from 14 to 35 Gy (mean 23.55 Gy). Results regarded 102 patients (mean follow-up 65.08 months). Complete remission was observed in all cases; 76 patients experienced a relapse after a mean interval of 22.03 months following radiotherapy, with a relapse-free rate of 22.82 % after 5 years. Extracutaneous progression was observed in 9 cases (8.82 %). An overall actuarial survival rate of 97.36 % was observed after 5 years. A salvage treatment of relapses brought 44 cases to remission. Although we have observed a relapse-free rate lower than that reported by others, dermatologic radiotherapy was "curative" in 25.49 % of cases. Such treatment appears particularly useful in the cases with few lesions and in the management of new cutaneous localizations of follicular center cell lymphoma.     

Recurrent localized primary cutaneous marginal-zone B cell lymphoma

A 36-year-old man with a prior diagnosis of primary cutaneous marginal-zone B cell lymphoma presented with newly-developed, small, erythematous papules and nodules on his upper left arm and pink-to-skin-colored, clustered papules on his left forearm. A biopsy specimen and immunohistochemical analysis of the left arm lesions showed a lymphocytic infiltrate which stained positively for CD20 and Bcl-2 and negatively for CD10. A PET-CT scan was negative for any extra-cutaneous manifestations of disease. These clinicopathologic findings are indicative of recurrent localized primary cutaneous marginal-zone B cell lymphoma.     

睾丸原发性弥漫性大B细胞淋巴瘤皮肤转移一例

患者男,74岁。右下肢暗红色斑块2个月,于2013年12月12日就诊于我科。2个月前患者无意中发现右下肢出现数个暗红色斑块,蚕豆大,无瘙痒、疼痛等不适,未予重视。随后斑块逐渐增大增多,部分有融合趋势。该患者曾于2013年9月因右侧睾丸无痛性肿大4个月于外院就诊,相关检查不详,诊断右侧睾丸肿瘤,行右侧睾丸根治性切除术……     

Primary cutaneous B cell lymphoma

A 54-year-old man, a road layer by occupation, presented with a 'leonine facies' and multiple tumors that were more commonly present over the exposed parts of the body. On investigation, he turned out to be a case of primary cutaneous B cell lymphoma with a distinctive histopathology. Chemotherapy was given with a good therapeutic response.     

Treatment of cutaneous T cell lymphoma

Primary cutaneous T cell lymphomas encompass a wide variety of lymphomas that are characterized by a distinct clinical presentation. Advanced biological techniques have allowed a more precise classification in recent years. Stage-adapted therapy is at present the best approach to treat cutaneous T cell lymphoma.     

原发性皮肤T淋巴细胞瘤的治疗选择

原发性皮肤T细胞淋巴瘤是一组原发于皮肤的淋巴细胞异常增殖性疾病,蕈样肉芽肿是其中最常见的类型.原发性皮肤T细胞淋巴瘤的治疗选择,主要取决于世界卫生组织-欧洲癌症研究和治疗组织-国际皮肤淋巴瘤学会制定的TNMB分期与分类标准,其他需要考虑的因素包括,治疗方法的有效率、起效与持续缓解时间,治疗耐受性与相关毒副作用,治疗实施的难易程度及治疗费用等.原发性皮肤T细胞淋巴瘤早期主张支持治疗和局部治疗为主,晚期则要进行联合化疗,或根据病情辅以免疫调节治疗.一些特异的细胞因子、单克隆抗体和融合毒素也逐步试用于治疗原发性皮肤T细胞淋巴瘤.     

原发性皮肤B细胞淋巴瘤的研究进展(二)

阐述了原发性皮肤边缘区淋巴瘤、免疫细胞瘤和小腿原发性大Ｂ细胞淋巴瘤的临床、组织病理学等特征、相关性及其与慢性Ｂ淋巴细胞白血病、原发性皮肤滤泡中心细胞性淋巴瘤和浆细胞瘤等鉴别诊断。     

Gene expression profiles of cutaneous B cell lymphoma

We studied gene expression profiles of 17 cutaneous B cell lymphomas that were collected with 4-6 mm skin punch biopsies. We also included tissue from two cases of mycosis fungoides, three normal skin biopsies, and three tonsils to create a framework for further interpretation. A hierarchical cluster algorithm was applied for data analysis. Our results indicate that small amounts of skin tissue can be used successfully to perform microarray analysis and result in distinct gene expression patterns. Duplicate specimens clustered together demonstrating a reproducible technique. Within the cutaneous B cell lymphoma specimens two specific B cell differentiation stage signatures of germinal center B cells and plasma cells could be identified. Primary cutaneous follicular and primary cutaneous diffuse large B cell lymphomas had a germinal center B cell signature, whereas a subset of marginal zone lymphomas demonstrated a plasma cell signature. Primary and secondary follicular B cell lymphoma of the skin were closely related, despite previously reported genetic and phenotypic differences. In contrast primary and secondary cutaneous diffuse large B cell lymphoma were less related to each other. This pilot study allows a first glance into the complex and unique microenvironment of B cell lymphomas of the skin and provides a basis for future studies, which may lead to the identification of potential histologic and prognostic markers as well as therapeutic targets.     

原发性皮肤间变性大细胞淋巴瘤一例

［摘要］报告1例原发性皮肤CD30+大细胞性淋巴瘤。右前臂皮肤红肿浸润、溃疡结痂1月,左侧头面部红肿浸润10天。右前臂皮损组织病理检查：真皮深层以及皮下脂肪组织间瘤细胞浸润,密集分布。瘤细胞胞质丰富,呈嗜酸性,部分呈空泡状,细胞核大,异型性明显,核仁明显,染色质呈粗颗粒状,并可见病理性核分裂象;有少量成熟淋巴细胞浸润。行免疫组化检查示： 瘤细胞CD45R0（+）、CD30（+）,CD68（－）、CD20（-）、EMA（-）及ALK（-）。诊断为原发性皮肤间变性大细胞淋巴瘤     

原发性皮肤间变性大细胞淋巴瘤1例

报告1例原发性皮肤间变性大细胞淋巴瘤。患者女,49岁。右小腿结节1年,溃烂5个月。皮损组织病理检查:真皮内有密集的淋巴样细胞浸润,瘤细胞大、核呈肾形或不规则形、核分裂像多见,免疫组化示瘤细胞约70?30阳性、约20?45Ro阳性,而CD3、CD20、MPO、TIA-1、ALK-1均为阴性。诊断为原发性皮肤间变性大细胞淋巴瘤。     

Treatment of cutaneous lymphoma with etretinate

Twelve patients with various types of lymphoma were treated with etretinate. The diagnosis included parapsoriasis en plaque, epidermotropic lymphoma (diffuse chronic erythroderma with atypical mononuclear cells, Sézary syndrome or MF tumours) and non-epidermotropic lymphoma. The patients received etretinate in a dose of 0.8 to 1.0 mg/kg/day for 2 to 14 months. No additional therapy was given. Patients with epidermotropic lymphomas stage I and II had a favourable clinical and histological response whereas those with deeply infiltrating tumours remained unresponsive. Patients with parapsoriasis en plaque and poikiloderma showed little response. Of the four patients who discontinued the treatment, three had recurrences after 3 to 4 months but one remained clear. The results obtained with etretinate may equal those obtained with more aggressive treatments.     

原发性皮肤CD30+间变性大细胞淋巴瘤临床及组织病理分析

目的：探讨原发性皮肤CD30 间变性大细胞淋巴瘤(ALCL)的临床及组织病理学特征。方法：对3例患者的皮损行组织病理学及免疫组化检查,观察原发性皮肤CD30 ALCL组织病理学特征及CD45、CD3、CD20、CD30、EMA、HMB45等抗体染色结果。结果：3例患者临床上均表现为皮下结节。组织病理学特征为肿瘤细胞异形性明显,呈多形性,胞质丰富,核大,核仁明显。CD30标记阳性,不表达T细胞标记CD45RO及CD3。结论：原发性皮肤CD30 ALCL是具有独特形态特点及临床特征的肿瘤,根据组织学特征及免疫组化CD30阳性,可与其他恶性肿瘤进行鉴别。     

Treatment of radiation-relapsing primary cutaneous B-cell lymphoma with an anti-CD20 monoclonal antibody

Primary cutaneous B cell lymphomas have a high recurrence rate after treatment with surgery and/or local radiation therapy. Two men are described in whom radiotherapy-relapsing cutaneous B-cell lymphomas were successfully treated with the monoclonal anti-CD20 antibody rituximab. Both patients had a complete response with no recurrence at follow-up at 17 and 24 months for the large B-cell lymphoma of the leg and the follicle centre cell lymphoma, respectively. These are two of the few cases in the literature showing that rituximab is an effective and well-tolerated treatment for radiotherapy-relapsing primary cutaneous B cell lymphoma.     

原发性皮肤间变性大细胞淋巴瘤

报告1例原发性皮肤间变性大细胞淋巴瘤.患者女,24岁.左手中指红斑33 d,根据皮损组织病理及免疫组化标记结果诊断为原发性皮肤间变性大细胞淋巴瘤.     

第二讲向表皮性皮肤T细胞淋巴瘤的按分期治疗

向表皮性皮肤T细胞淋巴瘤(epidermotropic cutaneous T-cell lymphomas,ECTCL)系恶性辅助T细胞疾病,常开始于皮肤而后累及骨髓、血中淋巴细胞、淋巴结和内脏器官[1],包括具有独特和重叠临床表现的蕈样肉芽肿(Mycosis fungoides,MF)和Sézary综合征(Sézary's syndrome,SS).近应用聚合酶链反应扩增T-细胞受体γ链基因重排,证明MF在早期即示克隆性T细胞成分系统性侵犯[2],SS患者血中恶性T细胞成分明显扩增[3].ECTCL的病因仍然不明确,病程一般缓慢,在不同发展阶段,生物行为也不一致.多年来已采用很多不同的治疗方法[4],但尚未找出可延长患者存活的治愈疗法和确实有效的标准治疗方案.不过,治疗措施应根据患者疾病的分期分类和年龄、一般健康情况以及对治疗的耐受性和可利用的治疗设备而定[4].兹阐述ECTCL的按分期治疗措施如下:对ECTCL已采用很多不同的治疗方法[4],但经过随机化对比试验(randomized trial)却很少.仅Kaye等对比了作为起始治疗之综合化疗[环磷酰胺(CTX)、阿霉素(ADM)、长春新碱(VCR)、依托泊苷(VP-16]加全皮肤电子束放射治疗(total skin electron beam radiation therapy,TSEBRT)与保守治疗的情况,证明前者缓解(R)率较高,但毒性较大.两者对患者存活相似,结论为保守治疗倾向于作为起始治疗.ECTCL的治疗计划常规视个体患者而定,但根据文献报道不同分期采用的不同治疗方法,可供参考.     

Primary cutaneous B cell lymphoma: Outcomes and treatment

This retrospective study documents six patients with primary cutaneous follicular centre cell lymphoma (FCCL) of the head and trunk. The hack was the most common site of presentation of the primary. Despite a good response to initial therapy, cutaneous relapses were common and one patient developed lymph node metastases. Intralesional steroids may be effective in controlling localized skin relapses of this B cell lymphoma. All patients are currently alive with two surviving over 5 years.