原发性皮肤CD30阳性大细胞淋巴瘤1例

报告1例原发性皮肤恶性淋巴瘤。患者，女，14岁，左侧面部进行性肿胀3年余。病理报告为原发性皮肤CD30阳性大细胞淋巴瘤（PCCD30^ -LCL)。免疫组化染色示瘤细胞CD30阳性、CD45RO、CD20阴性。采用COP方案及CHOP-R方案化疗各1次后，效果较好，后失访。     

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。     

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。     

LCA阴性的原发性皮肤CD30+间变性大细胞淋巴瘤

报告1例原发性皮肤CD30＋变性大细胞淋巴瘤。患者女，48岁。左肩胛区红斑3年，逐渐增大、增多。组织病理检查曾疑诊为皮脂腺癌、默克尔细胞癌，后诊断为黑素瘤，并行手术切除，最后确诊为原发性皮肤CD30＋间变性大细胞淋巴瘤。     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

原发性皮肤CD4^＋多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4^＋多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断：原发性皮肤CD4＋多形性小/中T细胞淋巴瘤.     

原发性皮肤间变性大细胞淋巴瘤1例并临床分析

原发性皮肤间变性大细胞淋巴瘤是临床少见的一种皮肤肿瘤。其临床特征为原发于老年人皮肤的红色结节或肿瘤,病理特征为CD30阳性大细胞的大量表达(>75%)。治疗采用减量CHOP方案,效果明显。     

T cell receptor-gamma gene analysis of CD30+ large atypical individual cells in CD30+ large primary cutaneous T cell lymphomas

The hallmark of primary cutaneous CD30+ large T cell lymphoma are large lymphoid tumor cells, at least 75% of which, by definition, must be positive for CD30. The relatively benign clinical course of this lymphoma type has been explained with CD30-induced apoptosis, on the assumption that expression of CD30 defines the tumor clone; however, this hypothesis has not been tested on the molecular level to date. In this study we analyzed CD30+ cells in four patients with primary cutaneous CD30+ large T cell lymphoma by single cell polymerase chain reaction of T cell receptor-gamma genes followed by sequencing. Here, we demonstrate that most of the large CD30+ atypical cells possessed identical T cell receptor-gamma gene rearrangements, indicative of clonal proliferation. Nevertheless, polyclonally rearranged T cells were present in all CD30+ samples studied. In addition, one patient showed a second clone in a separate biopsy and three of four patients showed chromosomal imbalances as revealed by comparative genomic hybridization. Taken together, our data suggest that the CD30+ population in primary cutaneous CD30+ large T cell lymphoma indeed contains the tumor clone, thus providing molecular support for a link between clinical course and CD30-related signaling. Importantly, however, CD30 expression does not define the tumor clone as bystander T cells, as well as occasional additional clones, are also present in this population.     

CD30+ lymphoproliferative disorder: primary cutaneous anaplastic large cell lymphoma followed by lymphomatoid papulosis

CD30+ large anaplastic lymphoid cells are seen in anaplastic large cell lymphoma (ALCL), and also in lymphomatoid papulosis (LyP) and other lymphoproliferative disorders. It can be difficult precisely to categorize these disorders with CD30+ cells. We report a case of primary cutaneous CD30+ ALCL with systemic metastases in whom the clinical disease subsequently evolved into LyP. The patient was initially administered cisplatin and etoposide and made a good response. Eighteen months later, recurrent, self-healing cutaneous small nodules appeared around the original tumour site without any systemic involvement. Histopathological examination of the recurrent lesions revealed infiltration with a mixture of cells that included neutrophils, eosinophils and CD30+ large anaplastic cells cytologically identical with those in the primary lesion. The anaplastic cells in both the primary and recurrent lesions were positive for monoclonal antibodies CD30, CD25 and a monoclonal antibody directed against the chimeric protein p80(NPM-ALK). These observations suggest the possibility that the ALCL and the subsequent LyP represent different clinical manifestations of proliferation of the same clone.     

Primary cutaneous CD4 positive small/medium T‐cell lymphoma with high proliferation index and CD30‐positive large lymphoid cells

Primary cutaneous CD4 positive small/medium pleomorphic T‐cell lymphoma (SMPTCL) represents a provisional subtype of primary cutaneous T‐cell lymphoma with indolent clinical course. A few aggressive fatal cases with increased proliferation rate and few infiltrating CD8 positive T‐cells have been reported. We describe a case of SMPTCL with an increased proliferation rate, admixed CD30‐positive large lymphoid cells, and few infiltrating CD8 positive T‐cells. The lymphoma cells were positive for CD3, CD4, CD2 and CD5, and negative for CD8. A subset of the lymphoma cells was positive for follicular helper T‐cell markers bcl‐6 and PD‐1. There were approximately 20% CD30‐positive large lymphoid cells, and Ki‐67 showed a moderately high proliferation rate (～40%), mostly in the large lymphoid cells. CD8 infiltrating T‐cells were few (<5%). The patient had an indolent disease with complete response to radiation therapy. To the best of our knowledge, this is the first reported case of SMPTCL with an increased proliferation rate and large CD30+ cells that followed an indolent clinical course.     

Primary cutaneous CD30 anaplastic large cell lymphoma with keratoacanthoma-like pseudocarcinomatous hyperplasia and marked eosinophilia and neutrophilia

BACKGROUND: Pseudocarcinomatous hyperplasia (PCH) and marked tissue neutrophilia have been observed in cutaneous CD30(+) anaplastic large cell lymphoma (ALCL) occasionally and may cause misdiagnosis. METHODS: An unusual case of CD30(+) ALCL of the skin resembling keratoacanthoma (KA) both clinically and pathologically was described. Histologic examination and immunostaining were performed. RESULTS: A 55-year-old woman presented with a 3-month history of an enlarging hyperkeratotic tumor on the forehead with a central keratinous plug. Microscopic examination showed epithelial hyperplasia resembling KA and a diffuse background infiltrate of large atypical lymphoid cells in the dermis. The atypical cells resembled epithelial cells and were obscured by a massive infiltrate of eosinophils and neutrophils. Immunostaining confirmed the presence of a diffuse infiltrate of CD30(+) cells; these cells were negative for CD45RO, CD20, CD15, epithelial membrane antigen, anaplastic lymphoma kinase-1, and cytokeratin. There was no evidence of extracutaneous involvement. The findings were consistent with primary cutaneous CD30(+) ALCL of null cell phenotype with KA-like epithelial hyperplasia and marked eosinophilia and neutrophilia. CONCLUSIONS: Our case illustrates that primary cutaneous ALCL may be associated with KA-like PCH and concurrent marked tissue eosinophilia and neutrophilia. Diagnosis in such cases is challenging both clinically and histologically because the large atypical lymphoid cells may easily be obscured by the massive infiltrates of eosinophils and neutrophils or confused with invasive squamous cell carcinoma.     

CD30 positive anaplastic large‐cell lymphoma mimicking Langerhans cell histiocytosis

The presence of CD 1a+ dendritic cells (DC) has been well described in T‐cell lymphoproliferative disorders, and the presence of large numbers of DCs has rarely been reported as a mimicker of Langerhans cell histiocytsis (LCH). We present the case of a 56‐year‐old female with a solitary nodule on the chin whose case was referred to our institution for confirmation of the diagnosis of LCH. Skin biopsy showed an ulcerated nodule containing a wedge‐shaped infiltrate comprised of large atypical cells and cells with prominent grooved nuclei. The constellation of histologic and immunologic features favored a CD30 lymphoproliferative disorder of T‐cell lineage even though there were accompanying numerous dendritic histiocytes and CD1a positive Langerhans cells. The sheets of CD30 positive atypical lymphoid cells which express T‐cell markers were consistent with CD30 positive lymphoproliferative disease and favor CD30 positive anaplastic large‐cell lymphoma (ALCL) over Langerhans histiocytosis. The absence of Anaplastic Lymphoma Kinase (ALK) staining favored a primary cutaneous origin. This case signifies a CD 30+ ALCL of the skin which histopathologically mimics a LCH. Ezra N, Van Dyke GS, Binder SW. CD30 positive anaplastic large‐cell lymphoma (ALCL) mimicking Langerhans cell histiocytosis (LCH).     

原发性皮肤CD30阳性淋巴组织增生性疾病临床病理分析

目的 探讨原发性皮肤CD30阳性淋巴组织增生性疾病的临床及病理学特征。方法 对4例淋巴瘤样丘疹病及5例原发性皮肤间变性大细胞淋巴瘤的临床、病理学特征及免疫组化表达进行分析。结果 淋巴瘤样丘疹病分为A、B、C三型,组织学上形成一个连续的谱系,A型见多形性间变性大细胞或Sternberg-Reed样细胞散在分布或小片状分布在多量炎性细胞背景中;B型类似蕈样肉芽肿病变,表现为真皮层较宽的淋巴细胞浸润带,其中散在少量小至中等异形脑回样淋巴细胞;C型以间变性大细胞弥漫分布为特征,但临床上可自行消退。原发性皮肤间变性大细胞以皮下结节或皮肤丘疹就诊,瘤细胞体积大,呈多形性、圆形或椭圆形,胞质丰富,嗜酸或呈双色性,核大,核仁明显。两组病变中的大细胞均特征性表达CD30,预后均较好。结论 原发性皮肤CD30阳性淋巴组织增生性疾病是一组预后较好的皮肤T细胞性淋巴瘤谱系,综合临床表现、组织病理改变、免疫组化有助于本病的诊断。     

A case of anaplastic large cell lymphoma of skeletal muscle

Anaplastic large cell lymphoma (ALCL) is a high grade non‐Hodgkin lymphoma (NHL) that is comprised of the malignant proliferation of large lymphoid cells, which express CD30. Primary ALCL of the skeletal muscle is extremely uncommon. A 51‐year‐old Japanese female presented at our hospital with a 2‐month history of severe pain and swelling of the right leg. A gallium‐67 SPECT/CT scan showed a large mass involving the skeletal muscles from the gluteus to femoris. A biopsy of the mass demonstrated diffuse infiltration of medium and large neoplastic cells with round or lobulated hyperchromatic pleomorphic nuclei. A subset of Reed‐Sternberg‐like cells was also identified. Immunohistochemically, the neoplastic cells were strongly positive for CD4 and CD30, but negative for CD3, CD8, anaplastic lymphoma kinase (ALK), CD20, CD79α, CD21 and CD23. Based on the histological examination, this patient was diagnosed to have ALK‐negative ALCL of the skeletal muscle. Further studies are needed to clarify the biological behavior of primary skeletal muscle ALCL.     

皮肤原发性间变性大细胞淋巴瘤—附1例报告

报道1例皮肤原发性间变性大细胞淋巴瘤。运用HE染色观察组织学形态及LCA、UCHL1、L26、CD30、EMA等抗体的免疫组化染色,以确定本例肿瘤的免疫表型。结果本例瘤细胞表达CD30、EMA及T细胞相关抗原。皮肤原发性间变性大细胞淋巴瘤临床经过缓慢,预后良好。