儿童淋巴瘤样丘疹病(C型)一例

患者男,9岁,因全身丘疹、脓疱、结痂、坏死8年余就诊.皮损组织病理结合免疫组化符合淋巴瘤样丘疹病(C型).淋巴瘤样丘疹病属于原发性皮肤CD30+淋巴增生性疾病,具有自限性,病因研究较少,组织病理学可分为A、B、C三型.临床上易误诊为急性痘疮样苔藓样糠疹,C型组织病理学与原发性皮肤CD30+间变性大细胞性淋巴瘤相似,故鉴别诊断尤为重要.     

淋巴瘤样丘疹病

淋巴瘤样丘疹病是原发性皮肤CD30＋淋巴增生性疾病病谱中的一员，临床表现类似急性痘疮样苔藓样糠疹，可分为经典型和局限型两型，病理学上则具有T细胞淋巴瘤的特点，通常分为三型，瘤细胞特征性表达CD30抗原。本病的病因和发病机制不明，临床和病理学上需和急性痘疮样苔藓样糠疹、原发性皮肤间变性大细胞淋巴瘤、蕈样肉芽肿、CD30T细胞假性淋巴瘤及炎症浸润性皮肤病伴CD30^＋间变性大细胞等疾病相鉴别。本病有白限性，预后良好，多数患者不需要特殊治疗。10％～20％患者可与其它淋巴血液系统恶性肿瘤伴发，故应注意对患者进行随访观察。     

皮肤CD30+淋巴增殖性疾病的发病机制研究进展

皮肤CD30+淋巴增殖性疾病是仅次于蕈样肉芽肿的第二大类最常见的皮肤T细胞淋巴瘤,其发病率占原发性皮肤T细胞淋巴瘤的25%左右。该病是一种病谱性的疾病,临床上包括淋巴瘤样丘疹病和原发性皮肤间变性大细胞淋巴瘤以及其中的中间类型。近年来对皮肤CD30+淋巴增殖性疾病组织病理类型的细化、发病的病因学、淋巴细胞凋亡异常以及基因异常的研究,为揭示皮肤CD30+淋巴增殖性疾病发病以及治疗提供了一些新的观点。     

CD30淋巴增生性疾病谱:淋巴瘤样丘疹病到间变性大细胞淋巴瘤

CD30淋巴增生性疾病包括淋巴瘤样丘疹病和一系列非何杰金淋巴瘤。为了揭示CD30淋巴增生性疾病谱,回顾分析了文献报告的173例成人皮肤、间变性大细胞CD30淋巴瘤的临床表现、组织学和免疫学改变、细胞遗传学和分子生物学标准,评价了该病的诊断、治疗及预后判定因素,最后讨论了淋巴瘤样丘疹病和皮肤间变性大细胞淋巴瘤之间的关系。     

淋巴瘤样丘疹病分型和治疗的研究进展

淋巴瘤样丘疹病是一种相对常见的皮肤淋巴细胞增生性疾病,临床多表现为反复发作的坏死性丘疹,愈后留有萎缩性瘢痕。淋巴瘤样丘疹病特异性地表达CD30标记,与原发皮肤间变性大细胞淋巴瘤为谱系性疾病。本综述简要介绍了淋巴瘤样丘疹病的基本分型以及特殊的临床、病理和免疫组化类型,指出了目前诊断方面尚存在的问题,并简要介绍了淋巴瘤样丘疹病的治疗方案。     

原发性皮肤CD30+淋巴细胞增殖性疾病进展

原发性皮肤CD30^＋淋巴细胞增殖性疾病包括原发性皮肤间变性大细胞淋巴瘤、淋巴瘤样丘疹病和一些临界性肿瘤，构成疾病谱系。这些疾病的共同特点是肿瘤细胞表达CD30抗原。这类疾病预后较好．故选择治疗方法时需权衡积极治疗的短期疗效与潜在副作用间的利弊关系。CD30^＋细胞也可在其他疾病中见到，CD30^＋免疫组化结果应结合临床综合分析。     

CD30与皮肤淋巴组织增生性疾病

CD30又称Ki-1抗原,为一种分子量为120k Da的跨膜细胞因子受体,属于肿瘤坏死因子受体家族,可以参与细胞活化和分化、信号转导及免疫激活等,其表达显示了细胞的活化状态。可表达CD30的原发于皮肤淋巴组织增生性疾病主要有淋巴瘤样丘疹病、原发性皮肤间变性大细胞淋巴瘤,少见的病例还有皮肤霍奇金淋巴瘤和蕈样肉芽肿大细胞转化。本文针对上述疾病做一综述,以利于对这类皮肤病变更好诊断、鉴别诊断及治疗。     

皮肤淋巴瘤第六讲非MF/SS皮肤恶性淋巴瘤及其治疗原则

阐述非蕈样肉芽肿/Sézary综合征皮肤恶性淋巴瘤包括原发性皮肤淋巴增殖性疾病(淋巴瘤样丘疹病、原发性皮肤CD30阳性大T细胞淋巴瘤)、CD30阴性皮肤大T细胞淋巴瘤、多形小/中等大T细胞淋巴瘤、皮下脂膜炎样T细胞淋巴瘤、鼻和鼻型自然杀伤/T细胞淋巴瘤以及原发性皮肤滤泡中心细胞性淋巴瘤、原发性皮肤边缘区淋巴瘤和小腿大B细胞淋巴瘤、富于T细胞的B细胞淋巴瘤与血管内大B细胞淋巴瘤的治疗。     

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。     

Primary cutaneous anaplastic large cell lymphoma manifested as multiple nodules and plaques on scrota:a case report

报告1例以阴囊多发结节斑块为表现的原发性皮肤间变性大细胞淋巴瘤.患者男,53岁.因阴囊结节6个月就诊.体格检查:阴囊多发暗红色结节斑块,伴溃疡形成,双侧腹股沟淋巴结肿大.皮损组织病理学检查示真皮弥漫淋巴样细胞浸润,瘤细胞偏大,核不规则;免疫病理检查示CD30(+)、CD8(+)、间变型淋巴瘤激酶(ALK)-1(-).复习相关文献,发生于生殖器部位的原发性皮肤间变性大细胞淋巴瘤国内未见报道,英文文献中仅有2例报道.     

A comparison of clinical, morphological and immunohistochemical features of lymphomatoid papulosis and primary cutaneous CD30(Ki-1)-positive anaplastic large cell lymphoma

The advent of immunoperoxidase technique on paraffin embedded tissue has identified a number of shared immunologic markers present in various lymphoproliferative cutaneous disorders. Two such disorders are the recently described primary cutaneous CD30-positive anaplastic large cell lymphoma (ALCL) and lymphomatoid papulosis; both entities are characterized by CD30-positive large atypical cells predominantly of T cell origin. We have compared the clinical, morphological and immunohistochemical features of 50 patients with lymphomatoid papulosis to a group of 27 patients with cutaneous CD30-positive ALCL. There are clear differences between the clinical presentation in these two diseases, and although both are characterized by similar atypical cells, the histologic pattern and distribution of atypical cells is sufficiently different to allow distinction and specific diagnosis based on hematoxylin and eosin stained sections supported by the immunohistochemical stains. In addition, both diseases are characterized by a long benign course, rarely complicated by development of lymphoreticular malignancy and invariably demonstrate CDSO(Ki-I) antigen positive large atypical cells.     

Primary cutaneous anaplastic CD30+ large cell lymphoma

Primary cutaneous anaplastic CD30+ large cell lymphoma (PCALCL) is part of the spectrum of primary cutaneous CD30+ lymphoproliferative disorders together with lymphomatoid papulosis. It affects mainly elderly patients and presents as skin nodules that tend to ulcerate. Histological and immunohistochemical study show the expression of CD30 antigen in more than 75 % of neoplastic cells. Currently it is considered a low grade lymphoma with favourable prognosis and good response to treatments such as local radiotherapy, methotrexate or surgery. We report a 93-year-old patient with ulcerated nodules in her right leg. Histological and immunohistochemical study confirmed the diagnosis of PCALCL, of non-B, non-T origin. The patient was treated with local radiotherapy with progressive resolution of skin nodules and absence of relapse at 6 months follow-up.     

Neutrophilenreiches, CD30+ anaplastisches T-Zell-Lymphom in Assoziation mit einer lymphomatoiden Papulose

The 2005 EORTC / WHO classification includes three CD30+ lymphoproliferative disorders: 1) primary cutaneous anaplastic large cell lymphoma, 2) lymphomatoid papulosis and 3) borderline cases. These entities may present with many different clinical appearances. Therefore, a precise differentiation among them often is impossible. We present a 40-year-old female who initially presented with a neutrophil-rich, anaplastic CD30+ T cell lymphoma followed by lymphomatoid papulosis.     

Disseminated CD8‐positive,CD30‐positive cutaneous lymphoproliferative eruption with overlapping features of mycosis fungoides and primary cutaneous anaplastic large cell lymphoma following remote solitary lesional presentation

CD8‐positive, CD30‐positive cutaneous lymphoproliferative disorders constitute a rare subset of T‐cell lymphoproliferative conditions, including variants of primary cutaneous anaplastic large cell lymphoma (ALCL), mycosis fungoides, lymphomatoid papulosis type D, cutaneous gamma‐delta T‐cell lymphoma and cutaneous peripheral T‐cell lymphoma. These entities share overlapping clinical, histopathologic and immunophenotypic features, presenting both a clinical and pathological diagnostic challenge. Presented here is a 73‐year‐old man with a disseminated, indolent CD30+, CD8+ cutaneous lymphoproliferative disorder with overlapping clinical and histopathological features of both mycosis fungoides and primary cutaneous ALCL, as well as features of lymphomatoid papulosis. To our knowledge, this is the first case of a generalized CD8+, CD30+ eruption with features of both mycosis fungoides and primary cutaneous ALCL arising following an episode of solitary primary cutaneous CD8‐positive ALCL.     

Primary cutaneous anaplastic large cell lymphoma

Primary cutaneous anaplastic large cell lymphoma (PC‐ALCL) is a CD30+ lymphoproliferative disorder (LPD) of the skin with a relatively good prognosis in the absence of high‐stage disease. CD30+ LPDs comprise approximately 25%‐30% of primary cutaneous lymphomas and as a group represent the second most common clonal T‐cell neoplasm of the skin behind mycosis fungoides. Diagnosis of PC‐ALCL relies strongly on clinicopathologic correlation given the potential morphologic, clinical and molecular overlap with the other cutaneous CD30+ LPD, lymphomatoid papulosis, and more aggressive hematolymphoid neoplasms.     

Distinct effects of CD30 and Fas signaling in cutaneous anaplastic lymphomas: a possible mechanism for disease progression

Lymphomatoid papulosis is part of a spectrum of CD30+ cutaneous lymphoproliferative disorders characterized by spontaneous tumor regression. The mechanism(s) of regression is unknown. In a recent study, a selective increase in CD30 ligand expression in regressing lesions of lymphomatoid papulosis and cutaneous CD30+ anaplastic large cell lymphoma was shown, suggesting that activation of the CD30 signaling pathway may be responsible for tumor regression, whereas no difference in Fas/Fas ligand expression was found between regressing and nonregressing lesions. Therefore we tested the effects of CD30 and Fas activation on three CD30+ cutaneous lymphoma cell lines (Mac-1, Mac-2 A, JK) derived from nonregressing tumors of two patients who had progressed from lymphomatoid papulosis to systemic anaplastic large cell lymphoma. To evaluate the effects of CD30 signaling, the cell lines were incubated with a CD30 agonistic antibody, HeFi-1. Proliferative responses, mitogen-activated protein kinase, and nuclear factor kappa B activities were determined with and without CD30 activation. Mac-1 and Mac-2 A showed increased proliferative responses to incubation with CD30 activating antibody, HeFi-1. Inhibition of the mitogen-activated protein kinase activity caused growth inhibition of the Mac-1, Mac-2 A, and JK cell lines. Activation of the Fas pathway induced apoptosis in all three cell lines. Taken together, these findings suggest that resistance to CD30-mediated growth inhibition provides a possible mechanism for escape of cutaneous anaplastic large cell lymphoma from tumor regression. Mitogen-activated protein kinase inhibitors are potential therapeutic agents for the treatment of advanced cutaneous anaplastic large cell lymphoma. J Invest Dermatol 115:1034-1040, 2000     

Cutaneous CD30 positive lymphoproliferative disorders with coexistent epithelial neoplasms: Report of two cases

Primary cutaneous large cell anaplastic lymphoma (C‐ALCL) and lymphomatoid papulosis (LyP) are cutaneous CD30+ lymphoproliferative disorders (CD30+ LPD). An association with CD30+ LPD and pseudoepitheliomatous hyperplasia has been recognized. Additionally, rare reports of epithelial neoplasms such as keratoacanthomas and squamous cell carcinomas (SCC) occurring in association with both C‐ALCL and LyP have been reported. We report two cases of CD30+ LPD with associated epithelial neoplasms; one patient with a primary cutaneous CD30+ LPD and SCC identified within the same lesion, and the other with a keratoacanthoma arising in a lesion of LyP. The pathogenesis of this association is poorly understood although various hypotheses exist. Awareness of the coexistence of these entities will avoid misdiagnosis and incorrect treatment.     

Primary cutaneous anaplastic large-cell lymphoma

Primary cutaneous anaplastic large-cell lymphoma (ALCL) is a form of cutaneous T-cell lymphoma that is characterized by solitary or localized nodules or plaques. Histopathologic features include a diffuse, non-epidermotropic infiltrate with cohesive sheets of large anaplastic CD30+ tumor cells. This entity must be distinguished from systemic ALCL with cutaneous involvement and lymphomatoid papulosis. Treatment modalities include clinical monitoring, radiation therapy, and surgical excision, with systemic chemotherapy reserved for disseminated or extracutaneous disease.     

Primary cutaneous CD30+ lymphoproliferative disorders

Primary cutaneous CD30⁺ lymphoproliferative disorders are the second most common group of cutaneous T‐cell lymphomas (CTCL) and include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large T‐cell lymphoma (cALCL). Both disease entities share overlapping clinical, histopathological, and molecular features, thus representing a spectrum of cutaneous CD30⁺ lymphoproliferative disorders. LyP may be distinguished from cALCL by clinicopathological correlation. In some patients, both diseases may coexist at initial diagnosis or develop over the course of the disease. Mycosis fungoides (MF), the most common CTCL, is not considered a primary cutaneous CD30⁺ lymphoproliferative disorder, but may occur in some LyP patients. In addition, LyP‐like lesions may develop in MF patients. However, this is frequently a manifestation of MF rather than a representation of two different disease entities. Caution also has to be taken in the setting of transformed MF with lesions expressing CD30, as they may be mistaken for either LyP or cALCL, resulting in an inadequate therapeutic approach.