原发性皮肤边缘区B细胞淋巴瘤

患者男,30岁.背部散在红色结节3年余.皮损组织病理检查:真皮内淋巴细胞样细胞呈片状岛屿状浸润增生,部分细胞可见异形性,表真皮间可见无浸润带,表皮大致正常.免疫组化结果:中心细胞样小淋巴细胞Bcl-2、CD20、CD79a、mum-1(++),Kap-pa:Lambda＞10;CD5、CD10、Bc1-6(-);结节周...     

Primary cutaneous marginal zone B-cell lymphoma in a patient with chronic lymphocytic leukaemia

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade malignant lymphoma that presents in the skin with no evidence of extracutaneous localization at diagnosis. We present an 80-year-old woman with B-cell chronic lymphocytic leukaemia (CLL) who developed multifocal PCMZL lesions 14 months after CLL diagnosis. PCMZL was clonally similar to the original bone marrow (BM) CLL cells. The specific translocation t(14;18) (q32;q21) with breakpoints in IGH and BCL2 loci was found in a skin specimen, but was absent in BM and peripheral blood (PB) cells. In contrast, a 13q deletion was found in BM and PB CLL cells. The patient was treated with chlorambucil and complete response of PCMZL was achieved. To our knowledge this is the first patient with CLL in whom PCMZL has been diagnosed.     

Complete resolution of primary cutaneous marginal zone B-cell lymphoma on the cheek of a 7-year-old boy with intralesional triamcinolone and tincture of time

A 7-year-old healthy boy presented with an asymptomatic smooth, firm red plaque on the cheek. Histopathology, immunostaining, molecular testing and imaging confirmed a diagnosis of a primary cutaneous marginal zone B-cell lymphoma. The lesion was treated with intralesional triamcinolone, with complete clinical resolution achieved within one year. Intralesional steroid injection is an effective first-line modality for the treatment of patients with limited disease in cosmetically sensitive areas.     

原发性皮肤边缘区B细胞淋巴瘤组织病理特征和免疫表型分析

目的：研究原发性皮肤边缘区B细胞淋巴瘤（primary cutaneous marginal zone lymphomas,PCMZLs）的临床及组织病理特点和免疫表型特征。方法：收集15例PCMZL,分析临床及组织病理特点,并用免疫组化检测CD21、BCL2、BCL10及B细胞标记CD20和CD79a等。结果：15例PCMZL中女9例、男6例,平均年龄61．2岁,中位年龄58岁。11例发生在头面部,其中1例为复发性,3例在背部,1例为全身多发。均表现为皮肤红斑性结节。苏木精-伊红染色为真皮内弥漫淋巴样细胞浸润,10例可见反应性淋巴滤泡或呈模糊的结节。免疫标记显示肿瘤细胞标记CD20（＋）,CD79a（＋）,BCL2（＋）,BCL10（＋）,5例CD43（＋）,CD5、CD10均（-）,CD21可显示淋巴滤泡形态。结论：PCMZL常见于头面部,老年女性多见,细胞形态学特点和BCL2和BCL10免疫组化染色对皮肤淋巴组织增牛病变有诊断和答别作用。     

Primary cutaneous B-cell lymphoma: a clinical, histological, phenotypic and genotypic study of 21 cases

The clinical, histological, phenotypic and genotypic features of 21 primary cutaneous B-cell lymphomas (CBCLs) have been investigated. The patients were 13 men and eight women aged 34-91 years (median 67) at diagnosis. Eighteen patients had localized disease, and three had multiple skin lesions at diagnosis. Twelve patients developed cutaneous or extracutaneous recurrences, and five died from malignant lymphoma 7-84 months (median 36) after diagnosis. Histological examination showed features of marginal zone/mucosa-associated lymphoid tissue (MALT)-type lymphoma in 12 cases. Three of these had transformed to diffuse large B-cell lymphoma (DLBCL) in relapse biopsies. The remaining cases were seven primary DLBCLs and two cases tentatively classified as follicle centre cell (FCC) lymphoma. The neoplastic B cells showed similar phenotypes and genotypes in most cases (CD20+, CD79+, CD5-, CD10-, cyclin D1-, bcl-2+, bcl-x-, bax-, t(14;18)-negative). p53 protein was expressed in five cases, and four harboured mis-sense or loss-of-function mutations in the p53 gene. Deletion or promoter region hypermethylation of the p16INK4a gene was detected in two patients with DLBCL. The level of retinoblastoma protein expression and the proliferative fraction were significantly higher in DLBCL (> 50%) than in MALT- or FCC-type lymphomas (< 10%). Features associated with an unfavourable prognosis were the presence of multiple skin lesions at diagnosis, transformation from MALT-type lymphoma to DLBCL, and possibly p16INK4a aberrations. It is concluded that most CBCLs are dissimilar from FCC lymphomas and seem to be more closely related to marginal zone/MALT-type lymphomas. It is also suggested that there are fundamental differences between DLBCL and other histological categories of CBCL, indicating that cutaneous DLBCL is a separate entity with an increased growth potential and genetic features similar to DLBCL originating in other anatomical sites.     

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。     

Primary cutaneous marginal zone B-cell lymphoma with anetoderma: spontaneous involution plus de novo clonal expansion

We describe a rare case of primary cutaneous marginal zone B-cell lymphoma (PCMZL) in a 33-year-old male affected by hepatitis B virus (HBV)-related chronic active hepatitis. The patient presented with multiple cutaneous papulonodular lesions characterized by polymorphic immunomorphology, with inverse pattern of immunoglobulin light chain restriction in two different lesions, coupled with mucin deposition and anetoderma in a third lesion. The involution of lesions of PCMZL with secondary anetoderma has been previously described, but this has not been reported in the context of multiple lesions showing differing immunoglobulin light chain restriction. We speculate that these findings may be secondary to a variable interaction between a chronic antigenic stimulus (i.e. HBV) and its specific immune response.     

Rituximab in cutaneous B-cell lymphoma: a report of two cases

We report two patients with primary cutaneous B-cell lymphoma who were treated with rituximab, a new anti-CD20 monoclonal antibody. The first patient, who had a diffuse large B-cell lymphoma of the lower leg, achieved an 85% improvement. The second patient, who had a primary cutaneous B-cell lymphoma, which had undergone high-grade transformation and systemic spread, achieved a minor response of approximately 30%. Both patients subsequently relapsed. The first patient achieved complete clearance with a second course of rituximab given with systemic chemotherapy, but again relapsed. Treatment with rituximab has been reported to produce response rates of 48% in relapsed systemic low-grade or follicular lymphoma, but there are no previous reports of the use of rituximab in primary cutaneous B-cell lymphoma.     

Primary cutaneous B-cell lymphoma associated with actinic prurigo

We describe two patients with a diagnosis of actinic prurigo who subsequently developed cutaneous B-cell lymphoma. This is the first report, to our knowledge, of this association. We propose that chronic antigenic stimulation by ultraviolet radiation, in the context of actinic prurigo, may have been causal in the development of these unusual lymphomas.     

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。     

Schnitzler syndrome associated with systemic marginal zone B-cell lymphoma

Schnitzler syndrome is a rare condition defined by chronic urticaria and monoclonal IgM gammopathy. Malignant evolution to Waldenström disease (WD) has been reported in several cases. We report a 49‐year‐old man who developed a marginal zone B‐cell lymphoma (MZL) 3 years after the beginning of Schnitzler syndrome. This is the first report of MZL in association with Schnitzler syndrome. IgM gammopathy is a clue for the diagnosis of Schnitzler syndrome. This condition needs to be closely monitored; it can precede the onset of an authentic lymphoproliferative disorder including WD and rarely MZL.     

Primary cutaneous marginal zone lymphoma associated with juxta‐articular fibrotic nodules in a teenager

Primary cutaneous marginal zone lymphoma (PCMZL) has rarely been reported in teenagers and is occasionally associated with Borrelia burgdorferi infection. Juxta‐articular fibrotic nodules represent a unique, localized fibrosing response to spirochete infections, namely Borreliosis. Herein, we report a 15‐year‐old healthy boy who presented with a 4‐year history of progressive acquisition of asymptomatic, erythematous nodules, ≤3 cm, beginning with his right forearm (3), then right arm (1) and lastly his right inner thigh (1). Biopsy showed PCMZL in three of five samples, and inflamed, fibrotic nodules, near the elbow in two. The bottom heavy lymphomatous nodules consisted of mostly small CD20+ CD43+ lymphocytes, some with plasmacytoid features. Mature plasma cells were lambda light chain restricted by in situ hybridization. The juxta‐articular fibrotic nodules were located in the deep dermis and subcutis, had peripheral plasma cell‐rich infiltrates, and showed nodular sclerosis (morphea profunda‐like) in one, and lamellar and angiocentric sclerosis in the other reminiscent of quiescent lesions of chronic localized fibrosing leukocytoclastic vasculitis. Immunohistochemistry for B. burgdorferi revealed rare positive organisms; however, polymerase chain reaction (PCR) and serology were negative for B. burgdorferi as were serologic and/or PCR assays for Bartonella henselae, Ba. quintana, Ehrlichia chaffeensis, Treponema pallidum, Helicobacter pylori and Babesia microti. No evidence of extracutaneous disease was found by the review of systems and imaging studies. A 4‐week trial of doxycycline therapy failed, whereas intralesional (IL) corticosteroid therapy induced rapid regression of his nodules. After two local recurrences, also treated with IL corticosteroids, he is well, without cutaneous disease, 20 months later. A literature review of 19 pediatric cases PCMZL reveals a similar natural history as adult PCMZL. Despite negative serology and PCR for B. burgdorferi, the occurrence of ipsilateral juxta‐articular fibrotic nodules, positive B. burgdorferi immunohistochemistry and rapid response to IL corticosteroids implicate the presence of a replicative or non‐replicative infectious (spirochetal) antigen in the initiation and promotion of this teenager's PCMZL.     

Primary cutaneous marginal zone lymphoma with subclinical cutaneous involvement and biclonality

Primary cutaneous extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) represents a monoclonal B-cell neoplasm that typically presents with papules, plaques or nodules. We describe a patient with a primary cutaneous MALT lymphoma with unusual clinical features and an unusual immunophenotype. Conventional microscopy together with immunohistochemistry and in-situ hybridization showed the presence of lymphoma in normal-appearing and minimally erythematous skin as well as in clinically involved skin. Furthermore, at least two distinct clones were shown, one of which had κ-light chain restriction, and the other of which had λ-light chain restriction. This case represents a newly described clinical appearance of primary cutaneous MZL and shows that some patients may have more than one neoplastic clone.     

Intralesional rituximab for cutaneous B-cell lymphoma

Rituximab, a chimeric anti-CD20 monoclonal antibody, has been approved for systemic treatment of relapsed or refractory CD20-positive B-cell non-Hodgkin's lymphoma. As cutaneous B-cell lymphoma (CBCL) also expresses the CD20 molecule, three patients with histologically and immunohistochemically confirmed CBCL without systemic involvement were treated with low-dose intralesional rituximab in a pilot study. Single doses applied ranged from 10 to 30 mg per lesion, according to lesion extent, with a cumulative dose of up to 350 mg. Injections were given two or three times weekly for 3-5 weeks, with a second cycle after 6 weeks in one patient with incomplete remission. Complete and lasting remission was achieved in each patient; this has persisted for up to more than 1 year. The observed adverse events were of grade 1 severity. Results suggest that intralesional rituximab may be a safe and effective new therapy modality for CBCL.