1例种痘水疱病样皮肤T细胞淋巴瘤患者的护理

<正>种痘水疱病样皮肤T细胞淋巴瘤(hydroa vacciniforme-like cutaneous T cell lymphoma)是近年来发现的一种少见的皮肤T细胞淋巴瘤,儿童多见。皮损部位以颜面和四肢皮肤曝光部位为主[1],也可累及躯干和臀部等非曝光部位,目前暂定为结外NK/T细胞淋巴瘤[2]。皮损呈多型性,可表现为水肿性红斑、水疱、溃疡和残毁性疤痕。可伴有发热、淋     

种痘水疱样皮肤T细胞淋巴瘤1例

种痘水疱样皮肤T细胞淋巴瘤( hydro vaceiniforme-like cutaneous T- cell lymphoma,HVLCTCL)是一种与EB病毒慢性活动性感染相关的罕见的T细胞淋巴瘤,复习国内文献,自2006年渠涛等1报道以来,迄今报道不过20例.由于2005年WHO/EORTC皮肤淋巴瘤分类中,本病被认为是结外NK/T细胞淋巴瘤(鼻型)的一个特型,2而未具体命名,故迄今国外仍有多种名称,如原发性皮肤EB病毒相关性T细胞淋巴增生性疾病(primary cutaneous Epstein - Barr virus - assci—ated T - cell lymphoproliferative disordes)、种痘水疱样淋巴瘤(hydro vacciniforme- like lymphoma)3、EBV相关性皮肤NK/T -细胞淋巴瘤(EBV - associated cutaneous NK/T- cell lymphoma)4、水疱样淋巴瘤( hydro - like lymphoma)5、种痘水疱样原发性皮肤CD8阳性T细胞淋巴瘤(hydro vacciniforme - like primary cutaneous CD8 - positive T- cell lynphoma)6、种痘样皮肤T细胞淋巴瘤7等.相广才等则习惯用牛痘样水疱病样皮肤T细胞淋巴瘤.8现将我们所见1例报道如下,并对迄今为止国内相关皮肤科文献报道的18例患者的临床组织病理、免疫组化及EB病毒检测等情况结合近期国外文献进行了复习.     

种痘样水疱病样皮肤T细胞淋巴瘤1例

种痘样水疱病样皮肤T细胞淋巴瘤是以皮肤水肿性红斑、水疱破溃遗留凹陷性瘢痕,同时伴有发热和肝脾肿大的一种罕见的皮肤T细胞淋巴瘤.该病多见于儿童,与Epstein-Barr病毒(EBV)感染相关.1现将我们诊断的1例,报道如下.     

伴面部严重肿胀的种痘样水疱病样皮肤T细胞淋巴瘤1例

报告1例伴面部严重肿胀的种痘样水疱病样皮肤T细胞淋巴瘤。患者男,23岁,面、颈、上胸部、双手背出现丘疹、丘疱疹、坏死、结痂、痘疮样瘢痕形成伴面部肿胀6年,加重4年。皮损组织病理及免疫组化检查符合种痘样水疱病样T细胞淋巴瘤。     

种痘样水疱病样淋巴组织增生性疾病研究进展

种痘样水疱病样淋巴组织增生性疾病是一种特殊的皮肤淋巴细胞增殖性疾病,与EB病毒感染有直接的关系。该病在东亚地区,包括日本、中国等地报告较多。种痘样水疱病样淋巴瘤容易发生在儿童,表现为面部、手背等光暴露部位为主的丘疱疹,愈后常遗留萎缩性瘢痕。患者常并发有系统表现,如发热、淋巴结增大,肝脾增大等,部分患者预后不良。种痘样水疱病样淋巴组织增生性疾病属于谱系性疾病,二者之间常无法严格区分,临床常见到种痘样水疱病转化为种痘样水疱病样淋巴瘤的病例。该文简要综述了种痘样水疱病样淋巴组织增生性疾病的临床和组织病理特点、发病机制以及治疗进展。     

种痘样水疱病样皮肤T细胞淋巴瘤

种痘样水疱病样皮肤T细胞淋巴瘤是近年来被逐渐认识到的一种与EB病毒相关的少见淋巴瘤,多见于儿童和青少年,主要累及面部和四肢,表现为丘疹、丘疱疹和水疱,随后出现坏死、溃疡、结痂,最后留下痘疮样瘢痕.本病临床表现独特,确诊主要依靠组织病理检查及免疫组化.目前尚无统一有效的治疗方案,报道的多数病例采用化疗,但疗效不一.     

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.     

种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤1例

患者女,17岁。全身反复起丘疹、水疱、坏死、凹陷状瘢痕伴瘙痒、发热15年,四肢起肿块2年。血清抗EBV-IgM(-),抗EBV-IgG(+)。肿块处皮损组织病理示真皮中下层和皮下组织见弥漫性致密的瘤细胞浸润,细胞核呈间变性;免疫组化示CD3(+),浸润的大细胞CD30(+),CD43(+),80%浸润细胞Ki-67(+)。水疱处皮损组织病理示表皮网状变性及多个水疱,真皮和皮下组织可见血管和附属器周围以淋巴细胞为主的、伴少量嗜酸粒细胞浸润,部分浸润细胞呈明显异形性;免疫组化示CD3(+),CD30(-),CD43(+),Ki-67(+)。诊断:种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤。确诊后建议患者转肿瘤科化疗,随访中。     

泛发型带状疱疹1例

报道1例非霍奇金淋巴瘤合并泛发型水痘-带状疱疹病毒(VZV)感染。患者男,51岁,左足红斑水疱疼痛0.5个月,皮疹1周;患者左足背、足踝处见红斑水疱,部分水疱破溃形成糜烂面,疼痛明显,全身泛发绿豆至黄豆大丘疱疹,部分可见脐凹。辅助检查:血三系降低,金黄色葡萄球菌:+;诊断:泛发型带状疱疹。该例患者有非霍奇金淋巴瘤,免疫力低下,加之不当湿敷,是造成水痘带状疱疹病毒全身泛发根本原因。     

水痘—带状疱疹病毒引起免疫抑制者巨大坏死性溃疡

患者女性,1914年11月21日生。因患有淋巴瘤伴颈部、腋部、膈下淋巴结肿大及骨髓、扁桃体受累而于1982年10月起给予联合化疗,以后改用瘤可宁治疗,1984年3月因骨髓抑制而停药。同年4月患者左鼻孔下出现单纯疱疹样水疱,但未作病毒学检查。至1984年5月,患者左侧上唇、鼻翼及邻近的上颌部出现直径约6cm的浸润肿胀,中央坏死,周围潮红并伴水疱及脓疱,右小腿亦见结痂性皮损一处,周围绕有水疱、脓疱,四肢有散在的丘疹水疱性皮损。面部及左小腿皮损处、反复多次进行病理活检,均符合病毒感染而不支持淋巴瘤的组织学变化。坏死部位多次取材均分离得水痘疱疹病毒。     

Lymphoma in patients with dermatitis herpetiformis and their first-degree relatives

BACKGROUND: The risk for lymphoma is increased in both dermatitis herpetiformis (DH) and in coeliac disease. The lymphoma most associated with coeliac disease is enteropathy-associated T-cell lymphoma. OBJECTIVES: To study the occurrence and type of lymphoma in a large series of patients with DH and their first-degree relatives. METHODS: The occurrence of lymphoma was studied in 1104 patients consecutively diagnosed with DH in two university hospitals during 1969-2001. A questionnaire was sent to 341 patients to examine the occurrence of lymphoma in their 1825 first-degree relatives. To analyse whether the DH patients with lymphoma had adhered to a gluten-free diet similarly to the patients without lymphoma, two age- and sex-matched patients with DH served as controls for each index case. Data on the gluten-free diet were collected from prospectively completed dietary forms and also from medical records. RESULTS: Eleven (1%) patients contracted lymphoma 2-31 years after the diagnosis of DH. Eight had B-cell-type lymphoma, two enteropathy-associated T-cell lymphoma and one remained unclassified due to missing material. Three (0.2%) of the first-degree relatives contracted lymphoma, all B-cell type. The 11 DH patients with lymphoma had adhered to a gluten-free diet significantly less strictly than the DH controls without lymphoma. CONCLUSIONS: The present study documents that patients with DH can have both B- and T-cell lymphoma. The DH patients with lymphoma had not adhered as strictly to the gluten-free diet as the control patients without lymphoma. The occurrence of lymphoma in the first-degree relatives was lower than in the patients with DH.     

Phenotypic heterogeneity of lymphoma of the skin

We studied surface markers present in 56 cases of lymphoma of the skin by immunohistochemical staining, using the ABC (avidin-biotin-peroxidase complex) and PAP (peroxidase-antiperoxidase complex) methods. Of these cases, 49 were T-cell lymphoma and 7 were B-cell lymphoma. Ten of the 49 cases of T-cell lymphoma were adult T-cell leukemia/lymphoma (ATL). Twenty-five of 31 cases of T-cell lymphoma except ATL analyzed by the ABC method showed a helper/inducer phenotype (Leu2a-,Leu3a+), two cases showed a suppressor/cytotoxic phenotype (Leu2a+, Leu3a-), one case showed Leu2a+Leu3a+, one case showed an inducer phenotype (Leu2a-, Leu3a+, Leu9+), and one case showed OKT11+, Leu2a-, Leu3a-, Leu1-, Leu9+, CD25+, Leu10+, CD30+. One CD8+ lymphoma was Pagetoid reticulosis, and a CD4+, CD8+ lymphoma was lymphomatoid papulosis with erythematous plaque. Cutaneous T-cell lymphoma (CTCL), previously described by Edelson et al., is defined as a helper T-cell lymphoma with marked affinity for the skin. In our study, 5 cases of T-cell lymphoma of the skin were not CTCL as described by Edelson et al. These results show that T-cell lymphoma of the skin is heterogeneous in nature. In other words, CTCL is one type but represents a major proportion of T-cell lymphomas of the skin.     

Cutaneous monomorphous CD4- and CD56-positive large-cell lymphoma

BACKGROUND: Recently, CD56 (NCAM)-positive lymphomas, such as nasal and nasal-type angiocentric NK/T cell lymphoma, aggressive NK cell leukemia/lymphoma and blastic NK cell lymphoma, were described by several authors as a unique group of lymphoma. OBJECTIVE: In this study, we intend to clarify the clinicopathological features of cutaneous CD4+ and CD56+ lymphoma. METHODS: Four patients with cutaneous CD4+ and CD56+ lymphoma were studied. RESULTS: Age at the first examination ranged from 71 to 89 years (mean = 81.2 years). One patient was female and 3 were males. The organ mainly involved at presentation was the skin. Lymphadenopathy, splenomegaly, leukemic spread and central nervous system involvement were observed as the disease progressed. The mean survival time was 12.2 months. Epstein-Barr virus was not detected within the tumor cells. CONCLUSION: This peculiar lymphoma is different from nasal and nasal-type angiocentric NK/T cell lymphoma and aggressive NK cell leukemia/lymphoma. Similar cases have been reported as blastic NK cell lymphoma/leukemia.     

Characteristics of cutaneous lymphomas in Korea

The clinicopathologic characteristics of malignant lymphomas vary according to geography. The aim of this study was to determine the relative frequency of cutaneous lymphomas and to examine the clinical relevance of the WHO classification in Korean cases of cutaneous lymphoma. The Korean Dermatopathology Research Group conducted a clinicopathologic review of a nationwide collection of 80 cutaneous lymphomas, diagnosed at 23 institutes over a recent 3-year period. The clinical records, haematoxylin & eosin-stained slides and immunohistochemical stains from 80 patients with malignant lymphomas of the skin were reviewed. In our study, the most frequent cutaneous lymphoma was mycosis fungoides. Compared with Western countries, Korea had higher rates of NK/T cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma and a much lower rate of B-cell lymphoma. The occurrence rates for various subtypes of malignant lymphoma in Korea are distinct from those in Western countries. The EORTC classification is not fully appropriate in dealing with Korean cases of cutaneous lymphoma, because NK/T cell lymphoma is not included in the EORTC classification for cutaneous lymphoma.     

Human herpesvirus 8-associated lymphoma mimicking cutaneous anaplastic large T-cell lymphoma in a patient with human immunodeficiency virus infection

Primary effusion lymphoma, a human herpesvirus 8 (HHV8)-associated lymphoma, is uncommon, and it is usually seen in human immunodeficiency virus (HIV)-infected patients. It presents as a body cavity-based lymphomatous effusion, but several cases of the so-called solid primary effusion lymphoma presenting as solid tumors without associated lymphomatous effusion have been reported. They have similar clinical, histopathological and immunophenotypical features. Most of them have a B-cell genotype. This suggests the solid variant may represent a clinicopathological spectrum of primary effusion lymphoma. We report a case of HHV8-associated lymphoma histopathologically and immunophenotypically mimicking cutaneous anaplastic large cell lymphoma. The patient was a 31-year-old HIV-seropositive man presenting with skin nodules over his right thigh. Biopsy of the nodules showed anaplastic large cells infiltrating the dermis. These malignant cells strongly expressed CD3, CD30 and CD43. Cutaneous anaplastic large T-cell lymphoma was initially diagnosed, but further tests, including immunoreactivity for HHV8 protein and clonal rearrangements of immunoglobulin genes, confirmed the diagnosis of HHV8-associated B-cell lymphoma with aberrant T-cell marker expression. This case provides an example of solid primary effusion lymphoma mimicking cutaneous anaplastic large T-cell lymphoma and highlights the importance of HHV8 immunohistochemistry and molecular tests in the diagnosis of HHV8-associated lymphoma with a cutaneous presentation.     

Lymphoepithelioid cell lymphoma (Lennert's lymphoma) presenting as atypical granuloma annulare

We describe a case presenting as atypical granuloma annulare where the underlying diagnosis, confirmed by lymph node biopsy, was lymphoepithelioid cell lymphoma (Lennert's lymphoma). Lennert's lymphoma is a peripheral T-cell lymphoma which may follow a variable course and transform into an aggressive phase. Cutaneous manifestations of this condition have only rarely been reported in the literature. The presence of granulomas in the skin may have either obscured the lymphoma infiltrate or may have reflected a more generalized immune response to the underlying malignancy.     

FHIT基因在原发性皮肤T细胞淋巴瘤中的异常改变及意义

目的检测原发性皮肤T细胞淋巴瘤中脆性组氨酸三联体(FHIT)基因的转录情况。方法用PCR-SS-CP法检测了10例原发性皮肤T细胞淋巴瘤患者皮损FHIT基因外显子5和8的缺失和突变状况,10例健康皮肤组织做对照。结果10例肿瘤组织中,FHIT基因外显子5缺失者7例,FHIT基因外显子8的缺失者2例;而正常人FHIT基因外显子5和8无1例缺失;对扩出的外显子进行SSCP分析,未检测到其突变。结论原发性皮肤T细胞淋巴瘤中存在FHIT基因外显子5和8的异常缺失,这种异常转录可能与该病的发生有关。     

Topical imiquimod as treatment for different kinds of cutaneous lymphoma

Imiquimod as a topical immune response modifier leads to a localized production of interferon and other cytokines. Apart from its use for genital warts it has therefore been used as treatment for different cutaneous neoplasms, including a few cases of cutaneous T-cell lymphoma. We treated 8 patients (4 with mycosis fungoides, 1 with CD30+ anaplastic large cell lymphoma and 3 with primary cutaneous B-cell lymphoma) with topical imiquimod. Therapy was started three times per week, in cases without response, the frequency was increased to a daily application. Two patients with mycosis fungoides and the patient with the CD30+ anaplastic large cell lymphoma had a complete clinical remission, the other two patients with mycosis fungoides did not show a response to imiquimod. Of the patients with cutaneous B-cell lymphoma, two reached a partial remission, one did not respond to therapy. Two patients had side effects such as erythema and pruritus which disappeared when the frequency of therapy was reduced. Our preliminary data show that imiquimod might be effective in some cases with therapy resistant lesions of cutaneous T-cell lymphoma as well as of cutaneous B-cell lymphoma, but more controlled studies are needed.     

36例皮肤天然杀伤细胞/T细胞淋巴瘤临床病理学研究

目的 探讨皮肤天然杀伤细胞(NK)/T细胞淋巴瘤临床病理学特点、与EB病毒的关系及预后。方法 收集2000—2010年北京大学医学部病理学系确诊为皮肤NK/T细胞淋巴瘤36例,分为原发与继发两组,分别观察临床病理学特点及与EB病毒的关系,并进行随访。结果 36例皮肤NK/T细胞淋巴瘤中,原发13例,继发20例,未能明确原发或继发3例。原发性与继发性皮肤NK/T细胞淋巴瘤均以男性好发,但两组男女性别比差异无统计学意义(P＞0.05)。与原发者相比,继发者发病年龄早(中位年龄,43.5比54岁,P＜ 0.05)、且临床上出现B症状(包括发热、盗汗或体质量下降)及多发皮损改变的频率较高(P值分别为＜0.05和＜0.01)。EB病毒在原发和继发病例中的检出率类似,分别为92.3％和85％。36例皮肤NK/T细胞淋巴瘤中位生存期为8个月,其中继发性皮肤NK/T细胞淋巴瘤中位生存期为6个月,明显短于原发者(18个月,x2= 6.074,P＜0.05)。结论 皮肤NK/T细胞淋巴瘤是一组与EB病毒密切相关、临床侵袭性强的肿瘤。但原发者较继发者发病年龄晚、预后较好。     

The spectrum of cutaneous lymphomas in Japan: a study of 62 cases based on the World Health Organization Classification

BACKGROUND: The relative incidence of malignant lymphoma subtypes differs according to geographic location. This study investigated the epidemiology of cutaneous lymphoma subtypes in Japan and compared it with other countries. METHODS: Sixty-two patients with cutaneous lymphoma attending the Department of Dermatology, National Hospital Organization Hokkaido Cancer Center were reviewed. The World Health Organization classification of hematopoietic and lymphoid malignancies was adopted. RESULTS: Of the 62 patients, 31 had primary cutaneous lymphoma (PCL) and 31 had secondary cutaneous lymphoma (SCL). T- and natural killer (NK)-cell lymphoma accounted for 80% of PCL, of which, mycosis fungoides accounted for almost 35%. Of the 31 patients with secondary cutaneous lymphoma, 17 patients (54%) had T- and NK-cell lymphoma, including nine adult T-cell leukemia/lymphoma patients, and 14 patients (46%) had B-cell lymphoma, including 11 diffuse large B-cell lymphoma patients. The majority of patients with SCL and NK-cell lymphoma with primary or secondary skin lesions had a poor outcome. CONCLUSIONS: PCL in this study showed a similar incidence to that of other institutions in Japan, while also demonstrating different frequencies from that of other countries, suggesting that the relative frequency of different PCL subtypes differ according to geographical location, similar to previous reports of systemic malignant lymphoma.