原发性皮肤弥漫大B细胞淋巴瘤（腿型）一例

报告1例原发性皮肤弥漫性大B细胞淋巴瘤（腿型）。患者男,51岁。右小腿出现多个红色结节5个月,组织病理检查：表皮和真皮之间见无细胞浸润带,真皮内淋巴样细胞浸润,细胞体积大,异形。肿瘤细胞CD20（++）,Bcl-6(+),Bcl-2(+),Pax-5(+),Ki-67 50％-75％(+),CD79a(+),MUM-1（+）,CD10（-）,诊断为原发性皮肤弥漫性大B细胞淋巴瘤（腿型）,给予CD20单克隆抗体加CHOP方案治疗,病情好转。     

原发性皮肤弥漫大B细胞淋巴瘤,腿型

报告1例原发性皮肤弥漫性大B细胞淋巴瘤（腿型）。患者女，82岁。左小腿出现3个红色结节2个月，组织病理检查发现真皮内淋巴样细胞浸润，无嗜表皮现象，细胞体积大，肿瘤细胞CD20（＋），CD79α（＋），Bcl-2（＋），Bcl-6（＋），Ki-67 70％（＋），MUM-1（＋），Pax-5（＋），CD10（-），诊断为原发性皮肤弥漫性大B细胞淋巴瘤（腿型），全身检查未发现皮肤以外系统受累证据，行局部肿瘤切除及口服糖皮质激素治疗。     

原发性皮肤弥漫性大B细胞淋巴瘤,腿型

报告1例原发性皮肤弥漫性大B细胞淋巴瘤,腿型.患者女,77岁.因右胫前多发暗红色斑块和结节3个月入院.皮损组织病理检查示:肿瘤细胞在真皮内弥漫性浸润性生长,侵及皮下组织,肿瘤细胞体积增大,形态不规则,细胞核大深染,核分裂象多见.免疫组化染色示:瘤细胞CD20(+),CD79a(+),Bcl-2(+),MuM-1(+).诊断:原发性皮肤弥漫性大B细胞淋巴瘤,腿型.通过CD20单克隆抗体加CHOP化疗方案治疗,病情明显好转.     

原发性皮肤弥漫性大B细胞淋巴瘤(腿型)一例

正原发性皮肤弥漫性大B细胞淋巴瘤(腿型)多见于老年女性患者,占原发性皮肤淋巴瘤的2%~4%,预后不良,临床极易误诊,现将临床误诊为皮肤结节病的原发性皮肤弥漫大B细胞淋巴瘤(腿型)1例报告如下。临床资料患者,女,68岁。主因左小腿内侧红色肿块伴疼痛3个月余,于2013年9月就诊。3个月前,无明显诱因患者左小腿胫侧皮下可触及3 cm×2 cm大     

原发性皮肤弥漫性大B细胞淋巴瘤一例

患者,男,51岁。左小腿红斑、结节、斑块伴疼痛3个月。组织病理及免疫组化确诊为原发性皮肤弥漫性大B细胞淋巴瘤,腿型。     

原发性皮肤弥漫性大B细胞淋巴瘤

报告1例原发性皮肤弥漫性大B细胞淋巴瘤.患者男,87岁.右下肢结节1年余.皮肤科检查:右下肢内侧可见8 cm×8 cm肿块,周围可见数个大小不等黄豆至蚕豆大的散在褐色结节.皮损组织病理示:表皮萎缩,真皮内可见单一核细胞呈团块状浸润,细胞体积大,胞质丰富,胞核淡染,并见核异形性和较多核分裂象.免疫组化结果:Bcl-2(+)、MUM-1(+).诊断:原发性皮肤弥漫性大B细胞淋巴瘤(腿型)(PCLBCL-LT).     

皮肤B细胞淋巴瘤的研究进展

 皮肤B细胞淋巴瘤是一组以皮肤损害为主要表现的成熟B细胞增殖性疾病,各亚型在临床表现、组织病理、免疫表型及遗传学特征具有异质性。其中原发性皮肤边缘区淋巴瘤和原发性皮肤滤泡中心淋巴瘤属于惰性淋巴瘤,原发性皮肤弥漫大B细胞淋巴瘤,腿型和血管内大B细胞淋巴瘤更具侵袭性,而EBV阳性皮肤黏膜溃疡呈惰性、自限性过程。惰性皮肤B细胞淋巴瘤首选局部治疗,侵袭性皮肤B细胞淋巴瘤首选利妥昔单抗联合化疗方案。本文从皮肤B细胞淋巴瘤的分类、临床表现、组织病理、免疫表型、遗传学特征、治疗和预后进行综述。     

原发性皮肤弥漫大B细胞淋巴瘤1例

报告1例原发性皮肤弥漫大B细胞淋巴瘤。患者男,72岁,因四肢、躯干红斑、结节、肿块2年,加重伴溃烂3月,于2009年3月9日就诊。皮损组织病理检查报告见皮肤真皮内大量淋巴样细胞弥漫浸润,未侵及表皮,免疫组化染色结果示瘤细胞L26（＋）,Bcl-6（＋）,Mum-1（＋）,CD10弱（＋）,K i-67（＋）约90%。诊断为原发性皮肤弥漫性大B细胞淋巴瘤。     

原发性皮肤弥漫性大B细胞淋巴瘤(腿型)2例报道伴文献复习

目的:通过探讨原发性皮肤弥漫性大B细胞淋巴瘤临床表现和病理特点,提高对该病的认识,实现早诊早治。方法:对2例疑似原发性弥漫性大B淋巴细胞瘤(腿型)患者进行临床表现分析及组织病理检查,通过检测细胞表面抗原标记确诊,采用利妥昔单抗联合CHOP方案化疗,21天为1个疗程,连续半年结束疗程继续随访。结果:弥漫性大B淋巴细胞瘤进展迅速,可破溃,组织病理检查表现为真皮单核细胞浸润,细胞异型明显,免疫组化表现为B细胞表型,CD20(+),CD79a(+),BCL-2(+),CD10(-),BCL-6(-),采用利妥昔单抗联合CHOP方案治疗,随访2年疗效肯定。结论:利妥昔单抗联合CHOP方案化疗连续6个疗程治疗原发性弥漫性大B细胞淋巴瘤,效果肯定。     

面部原发性皮肤弥漫性大B细胞淋巴瘤1例

原发性皮肤弥漫性大B 细胞淋巴瘤(primary cutaneous diffuselarge B-cell lymphomas,PCLBCL)是原发性皮肤淋巴瘤中比较少见的类型,被认为来源于生发中心或后生发中心,组织病理改变以中心母细胞和免疫母细胞浸润为主,见于老年患者的头、颈、躯干和四肢.国内文献报道发生于下肢的腿型PCLBCL相对多见, 而发生于头面部的PCLBCL 少见, 近期我科诊治1例,报告如下.     

Rituximab en el tratamiento de los linfomas cutáneos B primarios: revisión

Rituximab is a chimeric mouse-human antibody that targets the CD20 antigen, which is found in both normal and neoplastic B cells. In recent years, it has been increasingly used to treat cutaneous B-cell lymphoma and is now considered an alternative to classic treatment (radiotherapy and surgery) of 2 types of indolent lymphoma, namely, primary cutaneous follicle center lymphoma and primary cutaneous marginal zone B-cell lymphoma. Rituximab is also administered as an alternative to polychemotherapy in the treatment of primary cutaneous large B-cell lymphoma, leg type. Its use as an alternative drug led to it being administered intralesionally, with beneficial effects. In the present article, we review the literature published on the use of rituximab to treat primary cutaneous B-cell lymphoma.     

Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases

OBJECTIVES: To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. DESIGN: Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas. SETTING: Nineteen departments of dermatology in 10 regions of France. PATIENTS: Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas. MAIN OUTCOME MEASURES: Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Disease-specific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model. RESULTS: Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable short-term outcome. CONCLUSIONS: Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.     

Primary cutaneous B-cell lymphomas

Cutaneous B-cell lymphomas (CBCL) are the second most common form of primary cutaneous lymphomas. The cutaneous follicle center lymphoma and the cutaneous marginal zone lymphoma (extranodal MALT type lymphoma) account for the vast majority of CBCL and manifest with nodules. These two lymphoma entities have an indolent, slowly progressive course and an excellent prognosis despite a high rate of recurrences. In contrast, cutaneous diffuse large B-cell lymphoma, leg type, and other rare forms of CBCL display an impaired prognosis and therefore require to be treated with multiagent chemotherapy and anti-CD20 monoclonal antibodies in most cases. Clinico-pathologic correlation, histology with immunohistochemical profile and genotyping as well as staging examinations are crucial diagnostic elements in the work-up of CBCL.     

Linfoma cutáneo primario difuso de células grandes-tipo pierna con regresión espontánea

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT) accounts for approximately 20% of all primary cutaneous B-cell lymphomas and tends to present as infiltrated nodules, tumors, and plaques on the legs in the elderly. Unlike other primary cutaneous large B-cell lymphomas, it has a poor prognosis and tends to require treatment with systemic chemotherapy.We present the case of an 82-year-old patient with a 1-year history of nodules and plaques on her right leg. Biopsy led to a diagnosis of PCLBCL LT and the lesions resolved without treatment within 1 month of the first visit. This is an atypical course of PCLBCL LT and we believe that it is the first such case to be reported in the literature.     

大疱性类天疱疮并发原发性皮肤弥漫性大B细胞淋巴瘤

报告1例大疱性类天疱疮并发原发性皮肤弥漫性大B细胞淋巴瘤.患者男,80岁.全身水肿性红斑伴水疱、大疱,诊断为大疱性类天疱疮.在发病7个月后,右胫前出现多个暗紫红色结节,逐渐增多、增大,并扩展至右股内侧和背部.皮损组织病理检查示真皮全层及皮下脂肪小叶弥漫致密的淋巴细胞浸润,可见多数淋巴细胞体积增大,形态不规则,核大深染,可见核分裂相.免疫组化染色:CD20( ),bcl-2( ),bcl-6( ).诊断:原发性皮肤弥漫性大B细胞淋巴瘤.在肿瘤出现后1个月,患者大疱性类天疱疮的病情出现反复,通过CHOP方案治疗,病情明显好转.     

Comparative genomic hybridization analysis of cutaneous large B-cell lymphomas

The aim of the present study was to identify genetic aberrations in a series of patients with cutaneous large B-cell lymphoma (LBCL) using comparative genomic hybridization (CGH). Eighteen consecutive patients with primary (13 patients) (PCLBCL) and secondary (five patients) (SCLBCL) cutaneous large B-cell lymphoma were included in the study. Nine cases corresponded to PCLBCL leg type and four cases primary cutaneous follicle centre-cell lymphoma (PCFCL). Chromosomal imbalances (CIs) were detected in 14 of 18 samples (77.8%). All of nine cases with PCLBCL leg type and two of four cases with PCFCL showed CIs (100% and 50%, respectively). Regarding SCLBCL, in three of five cases (60%), CIs were detected. The most frequently detected gains involved 2q, 5q, 3 and 7q and amplifications affected 18, 12 and 13. Frequent losses were found in 17p. In PCLBCL leg type, the most frequent gains involved 2q and 7q, amplifications were localized in chromosomes 12, 13 and 18 and losses affected chromosomes 17p and 19. In PCFCL, gains located in 3q, 4 and 7q were found. Our study seems to confirm clear-cut differences between primary cutaneous LBCL and nodal diffuse LBCL, and it suggests the presence of genotypic differences between cases of PCLBCL leg type and cases of PCFCL.     

Bone involvement in two cases of thoracic primary cutaneous diffuse large B-cell lymphoma, leg type

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL-LT), is defined by a predominance of confluent sheets of centroblasts and immunoblasts, which strongly express Bcl-2 protein. This cutaneous lymphoma is mainly characterized by the development of skin lesions on the lower leg. Other localizations are possible (namely non-leg PCLBCL-LT) and usually affect younger patients. PCLBCL-LT is distinguished from the two other subtypes of primary cutaneous B-cell lymphomas by its immunohistopathological features, its aggressive clinical behaviour with a worse prognosis linked to skin recurrences, but also secondary extracutaneous spread. Bone involvement underlying skin lesions has been reported in few series and cases reports during PCLBCL-LT. We describe here two aggressive cases of PCLBCL-LT with high burden and infiltrative thoracic tumours, with localized bone involvement.     

The histomorphologic spectrum of primary cutaneous diffuse large B-cell lymphoma: a study of 79 cases

Primary cutaneous large B-cell lymphomas (PCLBCL) have historically been a matter of debate in the literature. The 2005 World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification scheme segregated cutaneous B-cell lymphomas into 3 groups: primary cutaneous marginal zone B-cell lymphoma, primary cutaneous follicle center cell lymphoma, and primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), "leg type" (PCDLBCL-LT). Additionally, the WHO-EORTC classification scheme utilized the term PCLBCL "other" not otherwise specified (NOS) type for rare cases of PCLBCL not belonging to either the "leg type" or the primary cutaneous follicle center cell lymphoma group. In this study, we retrospectively assessed the histomorphologic features of 79 cases of PCDLBCLs, including those of "leg type" and "other" NOS type, to further categorize the histologic spectrum of these unusual cutaneous neoplasms. The histologic diagnosis of PCLBCL usually poses little diagnostic difficulty; however, some cases may adopt unusual or unfamiliar appearances mimicking other lymphoproliferative disorders or other malignant neoplasms. Seventy-nine cases, occurring in 37 men and 42 women, aged 34-94 years, were analyzed. Fifty-three cases were classified as "leg type" and 26 cases were classified as "other" NOS type using the WHO-EORTC classification. Of the 53 cases classified as "leg type," 33 were women and 20 were men; of the 26 cases of "other" NOS type, 9 were women and 17 were men. In the "leg type" category, 31 cases were located on the lower extremities, 5 cases on the face, 5 cases on the arm, 3 cases on the chest, 2 cases on the shoulder, 2 cases on the back, 1 case on the trunk, 1 case on the buttock, 1 case on the supraclavicular area, 1 case on the head, and 1 case on the flank. Of the "other" NOS type category, 8 cases were located on the face, 5 cases on the shoulder, 3 cases on the head, 2 cases on the abdomen, 2 cases on the chest, 1 case on the trunk, 1 on the vulva, 1 on the axilla, 1 on the back, 1 on the neck, and 1 on the hip. Most cases assessed showed the classic morphologic appearance of PCDLBCL, but cases mimicking Burkitt lymphoma (starry-sky pattern), natural killer-cell (NK) lymphoma, mycosis fungoides (epidermotropism), low-grade B-cell lymphomas, epithelial malignancies, and Merkel cell carcinoma were encountered in this series. The high frequency of these rare histologic patterns can be explained by a bias associated with consultation practice. Careful histologic examination and immunohistochemical stains were used to establish the correct diagnosis. The differential diagnosis of PCDLBCL is broad and difficult to define histologically. Knowledge of these rare histologic variants is necessary to avoid misinterpretation of these cases as nonlymphoid malignancies.     

Systemische Therapie kutaner Lymphome

The management of patients with primary cutaneous lymphomas requires first of all a diagnostic work-up in order to differentiate between patients with nodal non-Hodgkin lymphomas with cutaneous manifestations and those with primary cutaneous lymphomas. In the latter an exact diagnosis of the type of cutaneous lymphoma has to be determined due to varying prognoses between the types of primary cutaneous lymphomas. In general, cutaneous lymphoma patients in early stages of disease can be treated successfully with skin-directed therapies. More advanced stages and some aggressive types like primary cutaneous diffuse large B-cell lymphoma, leg type or the leukemic variant of cutaneous T-cell lymphoma, the Sézary syndrome, require systemic treatment. The current recommendations regarding systemic therapy of patients with primary cutaneous lymphomas are reviewed.