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     

原发性皮肤弥漫大B细胞淋巴瘤(腿型)

报告1例原发性皮肤弥漫大B细胞淋巴瘤(腿型)。患者男,78岁。左小腿多个暗红色斑块、结节2个月余。左下肢皮损组织病理检查:真皮内弥漫淋巴样细胞浸润,肿瘤细胞体积大,胞质淡染,核大、不规则,异形性明显,有丝分裂象易见。免疫组化:CD3(-)、CD8(-)、CD20(+)、CD79α(+)、B细胞淋巴瘤(Bcl)-2(+)、Bcl-6(+)、多发性骨髓瘤癌基因(MUM)-1(-)、CD10(-)、T细胞内抗原(TIA)(-),Kappa(k)(+)、Lambda(λ)(-)、增殖核抗原(Ki-67)约95%(+)。诊断:原发性皮肤弥漫大B细胞淋巴瘤(腿型)。治疗上给予R-CDOP方案[利妥昔单抗+(环磷酰胺+脂质体多柔比星+长春新碱+泼尼松)]化疗后,左小腿肿块缩小。     

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。患者男,77岁。双小腿出现多个红褐色斑块和结节半年。皮损组织病理检查:肿瘤细胞无嗜表皮现象,表皮与真皮之间可见一条无细胞的透明带,真皮内可见较大的异形淋巴细胞样细胞浸润,肿瘤细胞体积大,大部分呈明显的圆形,细胞核大异形性明显,核分裂象易见。免疫组化:CD20(+)、CD79α(+)、B细胞淋巴瘤(B-cell lymphoma,Bcl)-2(+)、B细胞特异性激活蛋白(Pax)-5(+)、多发性骨髓瘤癌基因1(multiple myeloma oncogene 1,MUM1)(+)、肌酸激酶(CK)(-)、CD3(-)、Bcl-6(-)、CD10(-)、细胞周期素d1(Cyclin D-1)(-)、黑色素A(Melan A)(-)、CD7(-)、生长因子受体结合蛋白(Gr B)(-)、CD30(-)、CD56(-)、CD4(-)、CD8(-)及细胞核增殖抗原(Ki-67)约80%(+)。诊断:原发性皮肤弥漫性大B细胞性淋巴瘤,腿型。经过R-CHOP方案(环磷酰胺+表柔比星+长春新碱+泼尼松)化疗后,肿块明显退缩。     

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）二例并文献复习

弥漫性大B细胞淋巴瘤（腿型）是皮肤B细胞淋巴瘤的一个罕见亚型，具有特征性的临床、病理、免疫组化及演变特征。本文报道2例原发性皮肤弥漫性大B细胞淋巴瘤（腿型）并复习相关文献。     

早期呈丹毒样损害的原发性皮肤弥漫大B细胞淋巴瘤

患者男,69岁。右下肢红肿3个月,结节1个月余。患者早期右下肢皮肤红肿呈丹毒样损害,抗感染治疗欠佳,病程进展出现肿胀加重及多个紫红色结节。皮肤科检查:右下肢肿胀,可见多个紫红色结节,部分融合。右大腿及颈部散在豌豆大的红色或肤色结节。皮损组织病理检查:表、真皮可见无浸润带,真皮内及皮下脂肪层可见弥漫淋巴样细胞浸润,淋巴样细胞核大、深染,异形性明显,部分呈泡状核,有单个中位核仁,可见核分裂象。免疫组化:肿瘤细胞表达CD20、CD10、转录因子(MUM)-1、B细胞淋巴瘤/白血病(Bcl)-2;灶状表达Bcl-6;不表达CD3、CD5、CyclinD1及CD30;原癌基因(Cmyc)约70%阳性;核增殖抗原(Ki-67):阳性细胞约90%。诊断:原发皮肤弥漫大B细胞淋巴瘤(腿型)ⅢA期。予化疗后,皮损明显好转。     

Primary cutaneous B‐cell lymphoma in Nottinghamshire U.K.: prognosis of subtypes defined in the WHO‐EORTC classification

Background Primary cutaneous B‐cell lymphomas (PCBCL), with the exception of large B‐cell lymphoma of leg type and intravascular large B‐cell lymphoma, are associated with an excellent prognosis. These lymphomas have become much better understood in recent years leading to the publication in 2005 of the World Health Organization–European Organisation for Research and Treatment of Cancer classification. Objectives To determine the relative frequency of occurrence of subtypes of PCBCL in a defined population, and the survival of patients with these subtypes. Methods During the period 1987–2009, 61 consecutive patients with PCBCL were identified from the Nottingham Lymphoma Registry (population 1·1 million). After histological review, the number of patients with each subtype was as follows: marginal zone, 18; follicle centre, 14; diffuse large B cell, leg type, 16; diffuse large B cell, other sites, 12; and intravascular large B cell, one. Results The 5‐ and 10‐year lymphoma‐specific survival for patients with marginal zone lymphoma was 100%. The only patient with intravascular large B‐cell lymphoma died from widespread disease in spite of chemotherapy. The 4‐year lymphoma‐specific survival for follicle centre cell lymphoma was 90%. Patients with the other subtypes had the following 5‐year lymphoma‐specific survival rates: diffuse large B cell, leg type, 61% and diffuse large B cell, other, 40%. The median age at diagnosis for patients with diffuse large B‐cell lymphoma, leg type was 82 years and as a consequence the 5‐year overall survival was only 15%. There was a 3·4‐fold increase in the incidence of PCBCL from the period 1987–1997 to the period 1998–2009. Conclusions PCBCL is a rare disease (incidence around three per million population per year). It is, in our view, essential that it is diagnosed by a pathologist with an interest in cutaneous lymphoma and that the very different prognosis of the individual subtypes is appreciated by the treating clinician.     

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.     

弥漫性大B细胞淋巴瘤皮肤受累一例

患者,女,76岁,全身皮下多发结节伴右下肢皮肤溃烂2个月。皮损病理活检示（腹壁肿物）：非霍奇金淋巴结,B细胞型,免疫表型符合DLBCL,生发中心来源。免疫组化：CD20弥漫连片（+）,CD3散在（+）,CD10（+）,CD21显示FDC网消失,Ki67（80%）。胸腹部增强CT示胃窦十二指肠占位性病变,胃镜检查取胃窦部病变活检示：（胃窦小弯、大弯）非霍奇金B细胞淋巴瘤,考虑DLBCL或其他高侵袭性类型;免疫组化：粘膜腺体间弥漫的淋巴样细胞侵润性生长,CD20连片阳性,Ki67（90%）。我院血液科诊断为非霍奇金淋巴瘤（弥漫大B细胞型）IV期IPI,评分5分 继发皮肤受累。患者家属拒绝治疗,患者出院1周后死亡。     

大疱性类天疱疮合并原发性腹膜后弥漫大B细胞淋巴瘤1例

患者男,77岁。躯干、四肢反复红斑、水疱1年余,加重10d,确诊为大疱性类天疱疮。患者发病4年后体检时发现腹膜后肿物,外科手术切除肿物后组织病理显示淋巴样细胞弥漫性浸润,免疫组化示CD20(+),PAX-5(+),Bcl-6(灶+),MUM-1(+),Ki-67(90%+)。诊断:原发性腹膜后弥漫大B细胞淋巴瘤。     

Bcl-2, Bcl-6 and CD10 expression in cutaneous B-cell lymphoma: further support for a follicle centre cell origin and differential diagnostic significance

BACKGROUND: Primary cutaneous follicle centre cell lymphomas (PCFCCLs) are the most common type of cutaneous B-cell lymphoma. There is ongoing discussion on the origin of the neoplastic B cells in these PCFCCLs, and consequently on their relation to the groups of primary cutaneous marginal zone B-cell lymphomas (PCMZLs) and nodal follicular lymphomas. OBJECTIVES: To define better the neoplastic B cells in PCFCCLs, and to find out if differences in the expression of the antiapoptopic protein Bcl-2, and Bcl-6 and CD10, molecules which are normally expressed by the neoplastic B cells in nodal follicular lymphomas, might have diagnostic or prognostic significance in cutaneous B-cell lymphoproliferative disorders. METHODS: Pretreatment biopsies of well-defined groups of PCFCCL (n = 24), PCMZL (n = 14), primary cutaneous large B-cell lymphoma of the leg (PCLBCL-leg; n = 19), secondary cutaneous follicular lymphoma (n = 3) and cutaneous pseudo-B-cell lymphoma (n = 6) were investigated by immunohistochemistry for expression of Bcl-2, Bcl-6 and CD10. RESULTS: The PCFCCLs consistently expressed Bcl-6, whereas CD10 and Bcl-2 were expressed in only one and two of 24 cases, respectively. In contrast, PCMZLs were always negative for Bcl-6 and CD10, but were Bcl-2 positive, whereas skin and lymph node localizations of secondary cutaneous follicular lymphomas consistently expressed all of Bcl-2, Bcl-6 and CD10. Reactive follicle centre cells in pseudo-B-cell lymphomas expressed Bcl-6 (six of six cases) and CD10 (five of six cases), but not Bcl-2. PCLBCL-leg was Bcl-6 positive and CD10 negative in all cases, irrespective of clinical outcome, and strongly expressed Bcl-2 protein in all but two cases. CONCLUSIONS: The results of the present study provide further support for the follicle centre cell origin of both PCFCCL and PCLBCL-leg, and indicate that staining for Bcl-2, Bcl-6 and CD10 can serve as an important adjunct in the differential diagnosis of cutaneous B-cell lymphoproliferative disorders.     

Absence of the t(14;18) chromosomal translocation in primary cutaneous B-cell lymphoma

BACKGROUND: The t(14;18)(q32;q21) chromosomal translocation is found in the majority of nodal follicular lymphomas and in a lower percentage of systemic high-grade diffuse large B-cell lymphomas. The translocation results in the juxtaposition of the bcl-2 gene on chromosome 18 with the immunoglobulin heavy chain joining region on chromosome 14. Bcl-2 protein prevents apoptosis and the translocation leads to overexpression of a functionally normal Bcl-2 protein that prevents apoptosis of neoplastic cells. OBJECTIVES: The purpose of our study was to analyse cases of primary cutaneous B-cell lymphoma (PCBCL) for the presence of the t(14;18) translocation and to correlate the results with Bcl-2 expression and histological subtype. METHODS: Forty-four cutaneous B-cell lymphoid proliferations (36 PCBCL, four follicular B-cell lymphomas with cutaneous presentation and four reactive B-cell infiltrates) were analysed by polymerase chain reaction amplification and polyacrylamide gel electrophoresis using consensus primers for the joining region on the immunoglobulin heavy chain gene in combination with either a primer for the major breakpoint region (MBR) or the minor cluster region (mcr) on chromosome 18. RESULTS: None of 36 PCBCL analysed demonstrated a t(14;18) translocation; however, three of four systemic follicular B-cell lymphomas presenting in the skin were found to have a translocation in the MBR, which was confirmed by sequence analysis. Correlation with Bcl-2 immunostaining showed that of seven patients with high-grade cutaneous diffuse large B-cell lymphoma, four were Bcl-2 positive but had no evidence of a t(14;18) translocation. In the five cases classified as primary cutaneous follicle centre cell lymphoma, the neoplastic cells within the germinal centres failed to express Bcl-2. However, Bcl-2-positive neoplastic cells were present in all four cases of systemic follicular lymphoma, including the case that did not show a t(14;18) translocation. In all cases of marginal zone lymphoma the marginal zone lymphocytes were Bcl-2 positive. CONCLUSIONS: These findings indicate that the t(14;18) translocation does not occur in PCBCL, which suggests the involvement of different pathogenetic mechanisms compared with their nodal counterparts. Furthermore, the detection of a t(14;18) translocation in cutaneous B-cell lymphoma should suggest the presence of systemic disease, which underlies the need for exhaustive staging procedures.     

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.     

Composite cutaneous lymphoma of diffuse large B‐cell lymphoma‐leg type and subcutaneous panniculitis‐like T‐cell lymphoma

Composite lymphoma (CL) is a rare disease defined by the occurrence of two distinct lymphomas within a single tissue at the same time. We present the case of an 89‐year‐old male with a clinical history of immunoglobulin M monoclonal gammopathy of undetermined significance. The patient presented cutaneous eruption of nodules on the right bottom and arm. An excisional biopsy revealed cutaneous infiltration composed of two components. The first one consisted of large B‐cells with CD20+/MUM1+/BCL2+ phenotype whereas the second one involved the subcutaneous fat in a panniculitic manner, and was CD3+/CD8+/granzyme B+/TCRβF1+. The final diagnosis was CL of primary cutaneous large B‐cell lymphoma‐leg type (PCLBCL‐leg type) and subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL). We report and characterize for the first time coexistent PCLBCL‐leg type and SPTCL in a patient.