成人种痘样水疱病样EB病毒相关淋巴细胞增生性疾病三例

3例均为成年女性患者,全身可见对称性分布的红色丘疹、水疱、结节、坏死结痂及凹陷性瘢痕,伴有发热、淋巴结肿大等系统症状。组织病理学示:真皮内淋巴样细胞浸润,部分围绕血管周围浸润。血清中EB病毒抗体IgG阳性,外周血淋巴细胞有高拷贝的EB病毒DNA。患者外周血免疫分型示异常成熟T细胞增生。     

EBV相关的T/NK淋巴细胞增生性疾病1例

患者,女,6岁。全身反复红斑、水疱、坏死伴发热1年。系统症状:发热、全身多处浅表淋巴结肿大。皮肤组织病理检查:表皮内水疱形成,真皮全层、皮下脂肪层、血管周围、附属器周围可见灶状淋巴细胞浸润,部分淋巴样细胞轻度异形。免疫组化:CD_2(+)、CD_3(+)、CD_(43)(+)、TIA-1(+)、CD_4(+)。外周血EB病毒高载量。诊断为:EBV相关的T/NK淋巴细胞增生性疾病。     

种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病临床与病理分析

目的介绍、普及种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病的相关内容。方法回顾我科2014年诊断的3例儿童期发病的种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病,分析其临床与病理特点。结果 3例患儿临床特点为面部、上肢曝光部位反复发生水疱、丘疹、溃疡、坏死、结痂,后皮疹累及躯干、下肢等非曝光部位,留有萎缩性瘢痕。疾病呈慢性进展。3例均伴发热、肝脾淋巴结受累。辅助检查:3例血液EB病毒Ig G抗体(Epstein-Barr virus-Ig G antibody,EBV-Ig G)均阳性、EB病毒脱氧核糖核酸(Deoxyribonucleic acid,DNA)复制活跃。组织病理:真皮及脂肪层弥漫或灶状分布较密集的淋巴细胞浸润,细胞有轻度异型性。免疫组化:CD4、CD8、CD56阳性表达不一;Ki-67阳性比例低,TIA、Gram B散在阳性。EB病毒编码RNA原位杂交(EBV-encoded RNA,EBER)均阳性。TCR基因重排阴性。结论本病组织病理特异性不高、免疫组化标志尚无标准、TCR基因重排阳性率低。因此,特征性的临床表现和EB病毒病原学检验的诊断权重要高于组织病理、免疫组化、TCR基因重排。是否进展为种痘样水疱病样皮肤T细胞淋巴瘤需密切随访。     

非典型种痘样水疱病

报告1例非典型种痘样水疱病.患儿女,6岁8个月.反复发热、皮肤出现水疱4年,水疱愈合后遗留凹陷性瘢痕.皮肤科检查:面部、颈项部、臀部和四肢伸侧散在分布黄豆大凹陷性瘢痕,下肢可见数个暗紫红色浸润性斑块,压痛不明显,未见明显水疱.血清病毒学检测:EB(Epstein-Barr)病毒IgG抗体(EBV-cA-IgG Ab)(+).3年前曾行皮损组织病理检查:表皮水肿,水疱形成,真皮及皮下可见大量淋巴样细胞、中性粒细胞及少量嗜酸性粒细胞浸润,可见血管炎改变.皮损组织EBER原位杂交(+).诊断:非典型种痘样水疱病.     

种痘水疱病样皮肤T细胞淋巴瘤1例及文献复习

目的 报告1例种痘水疱病样皮肤T细胞淋巴瘤（HV-like CTCL）患者并复习该病临床、组织病理、分子生物学及治疗和预后特点,以提高临床医生对该病的诊治水平.方法 分析1例HV-like CTCL患者的临床病理、实验室检查及治疗预后特点,并复习近年国内外相关文献.结果 该患者表现为全身丘疹、丘疱疹、坏死、溃疡、结痂和痘疮样瘢痕等多形性皮疹,并伴有浅表淋巴结肿大.组织病理示真皮全层及脂肪小叶间隔密集的淋巴细胞浸润,细胞有异型性;免疫组化示肿瘤细胞表达CD2、CD3、CD8、TIAI、GramB,CD20,CD30,CD56阴性.皮损基因重排示TCR-δ（＋）.原位杂交检测Epstein-Barr（EB）病毒（＋）.患者经干扰素治疗病情缓解.结论 HV-like CTCL临床表现特殊,主要依靠组织病理及分子生物学手段确诊.目前该病没有统一的治疗方案,其预后与患病年龄、发病诱因（如血液EB病毒滴度、亚型和基因变异）及治疗等多种因素相关.     

儿童种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病的诊断与治疗3例

目的:探讨儿童种痘样水疱病样EB病毒(Epstein-Barr virus,EBV)感染相关淋巴细胞增生性疾病的诊断与治疗。方法:回顾性分析我科收治的3例种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病患儿的临床资料、实验室检查、治疗及预后等临床资料,并对相关文献进行复习。结果:3例患儿幼年发病,均因面部、四肢等部位皮损反复发作而就诊,其中2例伴有发热、肝大及淋巴结肿大。皮损组织病理检查均提示真皮浅层、皮下组织及毛囊附属器周围有大量淋巴细胞浸润,部分有轻度异形,免疫组化示CD43、CD56、Ki-67阳性表达不一;3例患儿血液EBV抗体阳性,EBV-DNA复制活跃。通过糖皮质激素、抗病毒等药物等治疗后,有2例病情明显缓解,1例效果不佳。结论:种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病与EB病毒感染密切相关,具有特征性临床表现,通过糖皮质激素、抗病毒药物联合静脉丙种球蛋白治疗部分有效,但仍有发展为种痘样水疱病样皮肤T细胞淋巴瘤可能,需密切随访。     

牛痘样水疱病样皮肤淋巴瘤与慢性活动性EB病毒感染的关系

目的 报道6例牛痘样水疱病样皮肤淋巴瘤,并研究其与慢性活动性EB病毒感染的关系.方法 临床病理分析、皮损免疫组织化学染色、血清学分析、EB病毒编码RNA原位杂交、外周血EB病毒DNA测定.结果 6例患者皮损均为反复发作的丘疹、丘疱疹、坏死、痘疮样瘢痕,其中4例还伴有程度不同的颜面、手足水肿.所有患儿均有长期间断发热等症状.皮损病理可见表皮多房性水疱,真皮全层大量淋巴细胞浸润,细胞形态异形,可见病理分裂象.4例皮损病理免疫组化染色,可见大量CD56阳性细胞,散在的CD3和CD45RO阳性细胞,T细胞内抗原-1和粒酶B染色阳性,诊断为牛痘样水疱病样皮肤NK/T细胞淋巴瘤;2例组化染色CD3和CD45RO阳性,CD56阴性,诊断为牛痘样水疱病样皮肤T细胞淋巴瘤.6例皮损均可见EB病毒编码RNA原位杂交阳性肿瘤细胞,血清学检查EB病毒衣壳抗原IgG抗体滴度升高,其中2例滴度为1:5120,2例为1:2560,2例为1:1280;2例患者外周血EB病毒DNA拷贝数高于正常.6例患儿均证实患有慢性活动性EB病毒感染.结论 牛痘样水疱病样皮肤淋巴瘤主要表现为颜面手足肿胀、水疱、痘疮样瘢痕,病理表现主要为真皮异形淋巴细胞浸润和血管中心坏死,免疫表型以NK/T型多见.慢性活动性EB病毒感染与该型淋巴瘤发病密切相关.     

种痘水疱病样成人EB病毒感染相关淋巴细胞增生性疾病

报告1例种痘水疱病样成人EB(Epstein-Barr)病毒感染相关淋巴细胞增生性疾病.该病是EB病毒感染诱发淋巴细胞增生引起的一系列临床疾病.皮损组织病理显示真皮全层淋巴细胞结节样浸润,呈楔形.结合文献对该病的发病机制、临床表现、诊断及转归进行复习.     

牛痘样水疱病与Epstein-Barr病毒潜伏感染的关系

牛痘样水疱病(hydroa vacciniforme)是一种较罕见的皮肤病,日光照射可诱发和加重皮肤损害。有研究发现,在牛痘样水疱病皮肤损害中,存在Epstein-Barr(EB)病毒感染的细胞,提示EB病毒潜伏感染可能与牛痘样水疱病的发病相关^[1]。本研究通过免疫组化、原位杂交、聚合酶链反应(PCR)方法分析牛痘样水疱病病理中浸润细胞的类型,并检测EB病毒的基因,以探讨牛痘样水疱病发病与EB病毒感染的关系。     

种痘水疱病样淋巴组织增生性疾病并发噬血细胞综合征

报告1例种痘水疱病样淋巴组织增生性疾病并发噬血细胞综合征。患儿男,13岁。因面部和四肢反复丘疹、丘疱疹、水疱及溃疡伴发热8年,加重2周入院。皮肤科检查：面部及四肢弥漫性肿胀,其上泛发暗红斑、丘疹、丘疱疹及溃疡,溃疡基底部覆有脓苔,其间可见大量痘疮样瘢痕;部分手指肌腱外露。实验室检查：血清Epstein-Barr病毒（EBV）拷贝数明显升高。左下肢皮损组织病理检查：大部分表、真皮坏死结痂,痂内及痂下方血管闭塞,管周淋巴细胞浸润,并可见个别病理性核分裂象。诊断：种痘水疱病样淋巴组织增生性疾病。入院后患儿存在持续发热,肝、脾大以及其他血液指标异常,提示并发嗜血细胞综合征。     

Human Ia-like-antigen-expressed cutaneous T cell lymphoma associated with Epstein-Barr virus infection

2 cases of cutaneous T cell lymphoma are reported. In both cases immunological studies revealed that the tumor cells from the cutaneous lesions had human Ia-like antigen on the cell surface, which was considered to be alloantigen primarily present on B lymphocytes. Serum examination showed an extremely high titer of two types of antibody against Epstein-Barr (EB) virus in the 1st case and a relatively high titer of one type of antibody in the 2nd case, suggesting that the patients had EB virus infection. Recently, some hematologists found the significant correlation of T lymphocytosis and the appearance of human Ia-like antigen in infectious mononucleosis. Based on these findings, it is assumed that EB virus has the ability of the immunological transformation of cutaneous neoplastic lymphocytes, and so we should not overlook the susceptibility of phenotypical changes of the original cell by EB virus when the immunological classification of cutaneous malignant lymphoma is done.     

Epstein-Barr (EB) virus infection in homosexual men in London

Twenty five homosexual men from London, 14 of whom had persistent lymphadenopathy and 11 of whom did not, were tested for immunity to Epstein-Barr (EB) virus. All yielded positive results to serological tests for the viral capsid antibody, and 11 had antibodies to the early antigen. Thirteen out of 17 were excreting virus into the saliva; culture of peripheral blood mononuclear cells from two of these patients showed no detectable regression induced by T cells that was specific to EB virus. No differences were found between the patients with and without lymphadenopathy. Peripheral blood B cells from six patients with hypergammaglobulinaemia were double stained for cytoplasmic immunoglobulin and EB viral nuclear antigen, and in all cases the activated B cells producing immunoglobulin did not contain EB nuclear antigen. Similarly, lymph node biopsy specimens from five patients showed no cells with EB nuclear antigen. These results indicate that although homosexual men have a high incidence of reactivated infection with EB virus, this viral infection is not the cause of the polyclonal activation of B cells seen in peripheral blood and is not implicated in the aetiology of the lymphadenopathy found in these men.     

Epstein-Barr (EB) virus infection in homosexual men in London.

Twenty five homosexual men from London, 14 of whom had persistent lymphadenopathy and 11 of whom did not, were tested for immunity to Epstein-Barr (EB) virus. All yielded positive results to serological tests for the viral capsid antibody, and 11 had antibodies to the early antigen. Thirteen out of 17 were excreting virus into the saliva; culture of peripheral blood mononuclear cells from two of these patients showed no detectable regression induced by T cells that was specific to EB virus. No differences were found between the patients with and without lymphadenopathy. Peripheral blood B cells from six patients with hypergammaglobulinaemia were double stained for cytoplasmic immunoglobulin and EB viral nuclear antigen, and in all cases the activated B cells producing immunoglobulin did not contain EB nuclear antigen. Similarly, lymph node biopsy specimens from five patients showed no cells with EB nuclear antigen. These results indicate that although homosexual men have a high incidence of reactivated infection with EB virus, this viral infection is not the cause of the polyclonal activation of B cells seen in peripheral blood and is not implicated in the aetiology of the lymphadenopathy found in these men.     

HSP-70在SLE中的表达及其与EB病毒感染的相关性

目的:探讨热休克蛋白-70(HSP-70)的表达与系统性红斑狼疮(SLE)发生、发展的关系及其与EB病毒感染的相关性。方法:采用逆转录聚合酶链反应(RT-PCR)方法,检测了60例SLE患者和30例正常对照组外周血单个核细胞中HSP-70的表达,酶联免疫吸附试验(ELISA)检测抗EB病毒核抗原IgG抗体的表达。结果:活动期患者HSP-70的表达明显高于稳定期组,稳定期组的表达明显高于对照组,其中活动期和稳定期患者中EB病毒感染阳性组HSP-70的表达均明显高于EB病毒阴性组。结论:EB病毒可能通过HSP-70的异常表达参与SLE的发病机制和病程。     

Primary cutaneous T-cell-rich B-cell lymphoma in a zosteriform distribution associated with Epstein-Barr virus infection

T-cell-rich B-cell lymphoma (TRBL) is a lately recognized B-cell lymphoma variant characterized by a minor population of neoplastic B cells existing in a background of predominant polyclonal T cells. We report an 86-year-old man with primary cutaneous TRBL associated with Epstein-Barr (EB) virus infection. Clinically, palpable scaly erythemas were distributed in a zosteriform pattern on the right abdomen. Histologically, massive cellular infiltrates were located in the upper- and mid-dermis. Higher magnification showed that the cellular infiltration was composed mainly of abnormal mononuclear, large lymphoid cells with clear cytoplasm and scattered mitoses and small lymphocytes, which represented in excess of 75% of all the infiltrating cells. Immunohistochemical staining revealed that the large cells were positive for the B cell marker, CD20, but negative for the T cell marker, CD3. On the other hand, the small cells were positive for CD3, but negative for CD20. Polymerase chain reaction (PCR) revealed EB virus DNA in the skin lesion. Primary cutaneous TRBL has only been reported in 15 cases worldwide. To our knowledge, this is the first case of primary cutaneous TRBL in a zosteriform distribution reported in the literature and the second case of primary cutaneous TRBL associated with the EB virus infection. We postulate that the EB virus may be a contributory pathogenetic event leading to monoclonal B-cell proliferation.     

Primary cutaneous T-cell lymphoma with an angiocentric growth pattern: association with Epstein-Barr virus

Summary We report a case of primary cutaneous T-cell lymphoma with an angiocentric growth pattern. The lesions had been confined for about 2.5 years to the skin, but there had been a gradual progression of the disease both clinically and histologically. We assessed the neoplastic clonality and the presence of Epstein-Barr (EB) virus genome in this case using immunohistochemistry. Southern blot analysis and RNA in situ hybridization. Clonal proliferation of a CD4⁺αβT-cell phenotype was demonstrated. In addition, the clonal population harboured the EB virus genome, which suggested that the virus was involved in the pathogenesis of the disease. The patient has remained in remission for 10 months, and has received treatment with cyclophosphamide and prednisone.     

银屑病患者血清抗EB病毒抗体的检测

目的：探讨EB病毒感染与银屑病的关系。方法：采用酶免疫法检测53例银屑病患者血清EB病毒抗壳抗原／IgG、IgM抗体，抗早期抗原－D／IgG抗体。抗核心抗原－1／IgG抗体。结果：银屑病患者血清抗ER病毒早期抗原－D／IgG抗体阳性率明显高于正常对照组（P＜0．001）。结论：银屑病患者体内EB病毒可能处于激活状态。     

Hydroa vacciniforme-like eruptions in a patient with chronic active EB virus infection

We report a case of chronic active Epstein-Barr (EB) virus infection (CAEBV) associated with skin eruptions mimicking hydroa vacciniforme (HV) in a 4-year-old boy. The patient had repeated episodes of vesiculo-necrotic eruptions on the face, scalp, and bilateral forearms one year before the first visit to our department. General symptoms including fever, hepatosplenomegaly, abnormal liver function, and cervical lymph node swelling were noted three months before the first visit. At the first visit, small, bean-sized, erythemic papules with central necrosis were observed on the face and anterior chest wall. Thumb-sized ulcers with crust were present on the bilateral forearms. Histopathological examination of an erythematous lesion in the submandibular area revealed parakeratosis with a thick crust, mild spongiosis in the epidermis, and a dense infiltration of lymphoid cells into the dermis and perivascular space. Laboratory examination showed EBNA x 40, EBV VCA IgG x 1,280, and EBV DNA (PCR) 8 x 10(4). EBV-encoded small nuclear RNA (EBER) positive cells were detected in the dermis by an in situ hybridization (ISH) method. Large granular lymphocytes (65%) with the NK cell phenotype were found in the peripheral blood. A real time PCR method showed 171,741 copies/ micro g DNA in CD 16 positive cells. Although latent EBV infection-associated eruptions have been documented, detailed skin manifestations in CAEBV are less well known.     

Hepatitis B antigen and antibody in a male homosexual population.

Sera from 600 male homosexual patients were tested for hepatitis B antigen (HBs Ag) and its antibody (HBs Ab). Thirty-one men (5-2%) were positive for HBs Ag. Testing for HBs Ab by immuno-osmoelectrophoresis 33 men (5-5%) were positive. However, sera of 85 patients negative for HBs Ab by routine methods were examined for HBs Ab by radioimmune assay. Thirty (35%) sera were found to be positive. No absolute correlation between the detection of HBs Ag, or previous history of hepatitis, jaundice, or current hepatitis was found. Similarly there was little correlation between presence of HBs Ab and this history. These observations suggest that the male homosexual population represents a pool of individuals within which the hepatitis B virus is readily transmitted, mainly as a subclinical infection although clinical hepatitis does occur in some patients. It is suggested that further work is necessary to determine whether the high antibody rate in male homosexuals is related more to sexual practice than to promiscuity.     

Gianotti-Crosti样发疹的传染性单核细胞增多症1例

患儿女,3岁,鼻塞、流涕、咽痛3 d,发热1 d入院治疗。入院第2天出现面部、臀部丘疹,逐渐累及四肢。实验室检查EB病毒IgM阳性;腹部彩超:脾脏增大。诊断:传染性单核细胞增多症,皮疹表现为Gianotti-Crosti样发疹。