皮肤B细胞性淋巴瘤临床病理及免疫组化研究

应用免疫组化方法对５例皮肤Ｂ细胞性淋巴瘤进行研究。５例分为四种亚型：皮肤免疫细胞性淋巴瘤，中心细胞性和中心母细胞性各１例，中心细胞－中心母细胞性２例。ＣＩＬ出现中心母细胞及瘤巨细胞，向高度恶性淋巴瘤转化，免疫组化显示：５例ＣＤ４５，ＣＤ２０阳性，ＣＤ４５Ｒｏ均阴性。１例ＣＣＢＬ又示κ、λ、ＩｇＧ，ＩｇＭ均呈阳性，为双标记阳性，探讨其发性机理，并将各亚型与皮肤Ｂ细胞性假性淋巴瘤及组织细胞性淋巴瘤进行     

A primary cutaneous multi-lobed B-cell lymphoma

Multi-lobed non-Hodgkin's lymphoma (NHL) has recently been recognized as a NHL variant. A patient presented with a scalp nodule which, upon skull X-Ray, was seen to be associated with a bone defect. Immunophenotyping clearly demonstrated that this was a B-cell proliferation. Histologically the B-lymphocytes were closely related to centroblasts. There were no other extra cutaneous localizations. The present report emphasizes the importance of this clinico anatomical entity which shows prominent extra-nodal involvement, large lymphoid cells with multi-lobed nuclei and a good response to chemotherapy. Multi-lobed NHL may be a T-cell lymphoma, or a B-cell lymphoma closely related to centroblastic NHL. Although multi-lobed lymphomas have a predilection for cutaneous localizations, our case is the first primary cutaneous multi-lobed B-NHL, proven by immunophenotyping.     

Juvenile onset of primary low-grade cutaneous B-cell lymphoma

Background  Cutaneous lymphomas rarely occur in children and adolescents, and are mostly of the T-cell lineage. Low-grade primary cutaneous B-cell lymphoma (CBCL) is extremely rare in individuals under 18 years old. Only 11 patients under 20 years old have been reported in the literature.
Objectives  To evaluate the number of patients younger than 18 years with primary CBCL diagnosed at our centre and to investigate its clinicopathological features, treatment and course in this age group.
Methods  We reviewed the files of all 90 patients with primary CBCL who attended the Department of Dermatology of our tertiary care university-affiliated centre from 1992 to 2007.
Results  Four patients who met study criteria were identified: three girls and one boy. Mean age at diagnosis was 16·6 years (range 16–17). Three patients had cutaneous marginal zone lymphoma (CMZL), and one had a spindle-cell (sarcomatoid) lymphoma, most probably follicular centre cell type. All were treated with the standard regimen used in adults. The mean duration of follow up was 45 months. No extracutaneous progression was noted. At present two of the four patients are in complete clinical remission.
Conclusions  In Israel, primary CBCL apparently occurs more often in young patients than reported in the literature. CMZL is the most frequent type. Long follow up is mandatory to assess the biological behaviour of CBCL in the paediatric/adolescent age group.     

皮肤原发性B淋巴母细胞淋巴瘤一例

患者男,56岁.2006年发现左前臂隆起性结节半年,渐增大.皮肤科检查:左前臂外侧可见一直径3 cm的肿物,其皮肤表面颜色略深,可见手术瘢痕长2.5 cm,肿物与皮肤粘连,固定,边界不清.组织病理学检查:瘤细胞圆形、卵圆形,小至中等大的淋巴母细胞,染色质细腻,核仁不明显,胞质少而淡染,弥漫分布.免疫组化:末端脱氧核苷酸转移酶(TDT)+、LCA+、CD20-、CD79a+、转录因子PAX5+、CD3-、CD30-、Ki-67阳性细胞数50％、上皮膜抗原(EMA)-、CD5-、CD10+、CD23-、CD56-.病理诊断:皮肤B淋巴母细胞淋巴瘤.治疗:给予环磷酰胺+多柔比星+长春新碱+泼尼松(CHOP)方案化疗,病情反复,随访4年9个月时患者死亡.     

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.     

原发性皮肤大B细胞淋巴瘤的研究进展

阐述原发性皮肤大B细胞淋巴瘤的分类、原发性皮肤富于T细胞的B细胞淋巴瘤、血管内大B细胞淋巴瘤和B-免疫母细胞性淋巴瘤的临床表现、组织病理变化、免疫组化、基因重排与诊断和鉴别诊断，以及皮肤大B细胞淋巴瘤的形态学变异的研究进展。     

Primary cutaneous B-cell lymphoma

Primary cutaneous B-cell lymphomas include extranodal marginal zone B-cell lymphoma, follicular lymphoma, large B-cell lymphoma, and, rarely, mantle cell lymphoma. Our purpose in conducting this review was to determine the clinical and behavioral characteristics of primary cutaneous B-cell lymphomas, their relationship to infectious triggers, and therapeutic response. We conducted a retrospective chart review of 23 adult patients presenting to the dermatology clinic at M. D. Anderson Cancer Center with primary cutaneous B-cell lymphoma between January 1999 and May 2003. Primary cutaneous B-cell lymphomas generally present on the head and neck, with the trunk and extremities afflicted to a lesser extent. Patients were found to have serologic evidence of prior infection with Borrelia burgdorferi (n = 10), Helicobacter pylori (n = 5), and Epstein-Barr virus (n = 6). Overall, treatment of primary cutaneous B-cell lymphoma should involve multiple modalities; however, specific treatment aimed at concurrent or suspected infection, particularly B burgdorferi, is a helpful adjunct and may achieve complete remission in a small subset of patients.     

Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma

In past years, association of primary cutaneous B-cell lymphoma (CBCL) with infection by Borrelia burgdorferi has been reported in a few patients. The evidence for a pathogenetic role was based on clinical grounds or raised titre of antibodies in serum. Both methods, however, do not prove the association between the micro-organism and the CBCL, especially in countries where infection by Borrelia burgdorferi is endemic. Moreover, the exact percentage of Borrelia burgdorferi-positive CBCL is not known. We retrieved from our files 50 cases of CBCL to perform PCR analysis of Borrelia burgdorferi DNA on paraffin-embedded tissue sections. Only patients with primary CBCL were selected. In all cases, monoclonality of the infiltrate was confirmed by immunohistological pattern of immunoglobulin light chains or molecular analysis of Jh gene rearrangement, or both. Specific DNA sequences of Borrelia burgdorferi-were identified in cutaneous lesions from 9 patients (follicle center lymphoma: 3/20; immunocytoma: 3/4; marginal zone B-cell lymphoma: 2/20; diffuse large B-cell lymphoma: 1/6). Specificity was confirmed by Southern blot hybridisation in all positive cases. We could show that Borrelia burgdorferi, DNA is present in skin lesions from a small proportion of patients (18%) with various types of CBCL. Our results may have therapeutic implications. In analogy to Helicobacter pylori-associated MALT-lymphomas, which in some cases can be cured by eradication of Helicobacter pylori infection, a proportion of CBCL may be cured with antibiotic therapy against Borrelia burgdorferi. Although yet speculative, adequate antibiotic treatment for patients with primary CBCL should be considered before more aggressive therapeutic options are applied, particularly in countries where infection by Borrelia burgdorferi is endemic. PCR analysis of Borrelia burgdorferi DNA is a fast test that should be performed in all patients with CBCL to identify those who more likely could benefit from an early antibiotic treatment.     

The differential diagnosis between primary cutaneous large B-cell lymphoma and cutaneous follicular lymphoma: prognostic and therapeutic implications

The differential diagnosis between primary cutaneous diffuse large B-cell lymphoma and cutaneous follicular lymphoma is one of the most difficult aspects of dermatopathology, even though morphological criteria are well established and a wide panel of antibodies is available to every laboratory. Such diagnosis is, however, not trivial because it has important prognostic and therapeutic implications. Nevertheless, when the literature is reviewed, there is a feeling that the diagnostic deficits from the past could perhaps be responsible for the differences observed in the therapeutic results with less aggressive treatments, such as rituximab. The current report briefly revises some cases of primary cutaneous diffuse large B-cell lymphoma treated with rituximab, which have been reported in the literature. It also presents an additional case emphasizing the current approach to the differential diagnosis.     

伴显著浆细胞分化的原发性皮肤边缘区B细胞淋巴瘤一例

患者男,65岁,因右胸皮疹1年,右肘关节皮疹5个月来我院就诊。患者1年前发现右胸皮下肿物,未予重视,5个月前发现右肘关节绿豆大小肿物,均无明显疼痛或瘙痒,逐渐变大,当地就诊考虑为皮肤良性肿物,嘱继续观察,肿物持续变大,遂至我院就诊……     

原发性皮肤的边缘区B细胞淋巴瘤的BCL10表达与染色体异常

目的 探讨原发性皮肤边缘区B细胞淋巴瘤（PCMZL）中BCL10蛋白的表达和相关染色体的异常。 方法 收集17例PCMZL，用免疫组化检测BCL10的表达，用荧光原位杂交（FISH）的方法分别检测API2-MALT1、BCL10、MALT1和IgH基因的异常。结果 在17例PCMZL中，BCL10的阳性率为94.1%（16/17），其中细胞质阳性率为64.7%（11/17），细胞核阳性率为29.4%（5/17）。在FISH检测中，所有病例都不存在t（11；18），t（1；14）和t（14；18）染色体异常。与其他部位的MALT淋巴瘤相比较，在PCMZL中染色体易位的发生率不常见，可能这些遗传学异常不是PCMZL发生中的重要因素，而有其他目前未知的因素参与肿瘤的发生。结论 BCL10的核表达与是否出现上述的染色体异常无关，是否代表更具侵袭性的一种预后标记，尚需长期随访观察。     

Borrelia burgdorferi-associated primary cutaneous marginal-zone B-cell lymphoma: a case report

An association between Borrelia burgdorferi with primary cutaneous B-cell lymphoma (PCBCL) has long been suspected but just recently, thanks to a polymerase chain reaction technique, it had been possible to demonstrate B. burgdorferi-specific DNA in skin lesions of patients with different PCBCL subtypes. Locating cases of PCBCL that are related to B. burgdorferi infection could be really important for therapeutic implications; in fact, there are several reports of PCBCL responding to antibiotic therapy against B. burgdorferi. We report a case of B. burgdorferi-associated primary cutaneous marginal-zone B-cell lymphoma that, after specific antimicrobial therapy, did not show any clinical regression. We can conclude that additional studies are necessary in order to establish the use of antimicrobial therapy in B. burgdorferi-associated PCBCL.     

Multilesional primary cutaneous diffuse large B-cell lymphoma responsive to antibiotic treatment

Borrelia burgdorferi infection has been implicated in cutaneous B-cell lymphoma. We report a case of multilesional primary cutaneous large B-cell lymphoma without extracutaneous spread in a patient with elevated B. burgdorferi titers. After antibiotic therapy, clinical remission and a subsequent drop in B. burgdorferi antibody titers were obtained.     

Borrelia burgdorferi-associated lymphocytoma cutis simulating a primary cutaneous large B-cell lymphoma

The distinction between primary cutaneous B-cell lymphoma and B-cell pseudolymphoma on a histologic basis may be difficult, particularly in some cases of Borrelia burgdorferi-associated lymphoid proliferations. We report two cases of B. burgdorferi-associated pseudolymphoma that showed a dense infiltrate with a predominance of large atypical B cells. Because of this misleading histologic feature, a diagnosis of primary cutaneous large B-cell lymphoma was first suspected in both cases. In one case, successive recurrences led to aggressive therapies before the B. burgdorferi infection was recognized. However, a detailed review of histologic and immunohistochemical features was finally suggestive of a B. burgdorferi-associated pseudolymphoma in both cases. The etiologic role of B. burgdorferi was confirmed by serology, polymerase chain reaction analysis of B. burgdorferi DNA within the lesional skin, and response to antibiotic therapy. Because the distinction between B. burgdorferi-associated pseudolymphoma and primary cutaneous B-cell lymphomas may be difficult and true B. burgdorferi-associated B-cell lymphomas have been described, we suggest that antibiotic therapy should be considered as a first-line treatment in suspected or confirmed cases of primary cutaneous B-cell lymphoma in regions with endemic B. burgdorferi infection.