Characterization of skin blister fluids from children with Epstein–Barr virus‐associated lymphoproliferative disease

Epstein–Barr virus (EBV)‐associated T‐ or natural killer (NK)‐cell lymphoproliferative disease (LPD) is a heterogeneous group of disorders characterized by chronic proliferation of EBV‐infected lymphocytes. Patients may present with severe skin manifestations, including hypersensitivity to mosquito bites (HMB) and hydroa vacciniforme (HV)‐like eruption, which are characterized by blister formation and necrotic ulceration. Skin biopsy specimens show inflammatory reactions comprising EBV‐infected lymphocytes. However, blister fluids have not been fully assessed in patients with this disease. Blister fluids were collected from three patients with EBV‐associated LPD: two with HMB and one with HV. Immunophenotyping of blister lymphocytes and measurement of tumor necrosis factor (TNF)‐α in blister fluids were performed. The patients with HMB and HV exhibited markedly increased percentages of NK and γδ T cells, respectively, in both peripheral blood and blister fluids. These NK and γδ T cells strongly expressed the activation marker human leukocyte antigen‐DR and were considered to be cellular targets of EBV infections. TNF‐α was highly elevated in all blister fluids. Severe local skin reactions of EBV‐associated LPD may be associated with infiltrating EBV‐infected lymphocytes and a high TNF‐α concentration in blister fluids.     

Quality of life assessment and disease experience of patient members of a web-based hydroa vacciniforme support group

Background/Purpose: Hydroa vacciniforme (HV) is a rare photodermatosis that primarily affects children. It is characterized by photodistributed vesicles that heal with scarring. The purposes of this study are to perform the initial investigation into the effect of HV on quality of life (QoL) and gain insight into disease diagnosis and management.
Methods: Using the listserv from a web-based, international HV support group, either the Dermatology Life Quality Index (DLQI) or the Children's DLQI (CDLQI), and an HV-specific questionnaire were administered.
Results: Fifteen HV patients participated, nine (60%) males and six (40%) females. Median age at onset was 7 years, and 11/15 (73%) were younger than 18 years. The majority of patients were Caucasian (73%). Children cited life quality as being negatively impacted by an inability to play outdoors while adults noted QoL influences due to limitations on clothing choices. The mean CLDLQI and DLQI scores, 12.1 and 8.5, respectively, suggest a higher negative QoL impact than previously reported indices for generalized eczema, atopic dermatitis, and psoriasis.
Conclusion: When compared with other dermatoses, HV appears to have an equal or greater impact on patients' QoL. Dermatologists should be aware of the psychosociologic impact of this disease, especially on young HV patients.     

Familial hydroa vacciniforme

Hydroa vacciniforme is a rare, idiopathic photodermatosis with an onset in childhood and characterized by acute vesiculation, crusting and scarring following sun exposure. Familial cases are extremely rare with only one previous report. We report a brother and sister, both of whom have developed hydroa vacciniforme.     

Factors influencing the photo-reproduction of hydroa vacciniforme lesions

Hydroa vacciniforme vesicles can be reproduced experimentaly with repetitive UVA irradiations, but this photo-reproduction is not constant. The aim of this study was to search for the factors that influence photo-reproduction. To reproduce hydroa vacciniforme lesions six patients underwent repetitive UVA and polychromatic irradiations on the back. In four patients out of six, UVA irradiation with high doses induced papulo-vesicular lesions. Photo-induced lesions were very close to those induced by sunlight. Photo-reproduction failed when phototesting was done after or shortly before remission. Therefore, the absence of photo-reproduction appears to be a good prognostic factor.     

异基因造血干细胞移植联合供者源淋巴细胞及EBV特异性杀伤T细胞治疗种痘水疱样淋巴瘤一例报告及文献复习

种痘水疱样淋巴瘤临床表现为疱疹,伴有发热、肝脾肿大、淋巴结肿大等系统症状。病理示真皮内淋巴细胞浸润,可见部分异形细胞,免疫组化示Bcl-6、Bcl-2、CD3、CD8、TIA、CD4、granzyme和EBER均阳性,而CD56、CD20均阴性。骨髓细胞TCR重排阳性。种痘水疱样淋巴瘤较罕见,临床易误诊。传统治疗效果差,异基因造血干细胞移植联合回输淋巴细胞及EBV特异性杀伤T细胞治疗种痘水疱样淋巴瘤的疗效好,可能是根治HVLL的有效手段。     

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。     

种痘水疱病样淋巴瘤2例临床病理分析

目的探讨种痘水疱病样淋巴瘤(hydroa vacciniforme-like lymphoma,HVLL)的临床病理学特点、诊断及鉴别诊断。方法对2例HVLL进行临床资料分析、病理形态观察、免疫组化和EB病毒原位杂交检测、T细胞受体基因重排检测。结果 2例患者分别为15岁和18岁,均有10年以上的反复头部种痘水疱病样皮疹伴高热。肿瘤细胞浸润真皮或至皮下组织,小到中等大,轻中度异型性,围绕皮肤附件和嗜血管生长。2例肿瘤细胞CD2、CD3、CD7、CD8、TIA-1和Granzyme B均(+);CD56和CD30均(-)。2例EBER均(+)。例1标本TCR克隆性重排检测(+)。例1患者行CEOD方案化疗1次,确诊后1年因肺部感染、呼吸循环衰竭死亡。例2患者行化疗4次,病情得以控制。结论 HVLL是一种罕见的发生于儿童的EB病毒相关性T细胞淋巴瘤,临床病史尤其是皮损特点为诊断重要提示,结合病理形态、免疫表型、EB病毒原位杂交、T细胞受体基因重排可确诊。     

种痘水疱病样皮肤T细胞淋巴瘤1例报道及文献复习

目的：探讨种痘水疱病样皮肤T细胞淋巴瘤的诊断与治疗,提高对该病的认识。方法：对1例种痘水疱病样皮肤T细胞淋巴瘤患儿的临床表现、辅助检查、诊疗过程进行分析,并复习相关文献。结果：该患儿主要表现为皮疹、发热、淋巴结肿大。皮疹呈多形性,表现为水肿性红斑、水疱、结痂、坏死及凹陷瘢痕,冬轻夏重,主要累及面部和四肢。EB（Epstein-Barr）病毒IgM抗体阳性。组织病理示皮肤真皮脂膜组织局灶性淋巴细胞浸润,以血管周围为主,免疫组化CD3 (+)、 CD43 (+)、 CD20 (-)、pax-5 (-)、TIA (+)、CD5 (+)、 CD8 (+)、 Granmye (+)、 CD4 (-)。激素治疗后病情缓解。结论：种痘水疱病样皮肤T细胞淋巴瘤的临床表现独特,确诊主要靠病损部位组织病理活检及免疫组化,部分病例对激素治疗反应良好,发病可能与EB病毒感染有关,预后有待于进一步观察。［中国当代儿科杂志,2009,11（7）：596-598］     

Umbilical Cord Blood as an Alternative Source of Reduced-Intensity Hematopoietic Stem Cell Transplantation for Chronic Epstein-Barr Virus–Associated T or Natural Killer Cell Lymphoproliferative Diseases

Chronic Epstein-Barr virus–associated T/natural killer cell lymphoproliferative diseases represented by chronic active Epstein-Barr virus infection are lethal but are curable with several courses of chemotherapy and allogeneic hematopoietic stem cell transplantation (HSCT). Recently, we reported that reduced-intensity conditioning (RIC) provided better outcomes than myeloablative conditioning because RIC was less toxic. However, it was unclear whether cord blood transplantation (CBT) works in the context of RIC. We retrospectively analyzed 17 patients who underwent RIC followed by bone marrow transplantation (RIC-BMT) and 15 patients who underwent RIC followed by CBT (RIC-CBT). The representative regimen was fludarabine and melphalan based. The overall survival rates with RIC-BMT and RIC-CBT were 92.9% ± 6.9% and 93.3% ± 6.4%, respectively (P = .87). One patient died of lung graft-versus-host disease after RIC-BMT, and 1 patient died of multiple viral infections after RIC-CBT. Although cytotoxic chemotherapy was also immunosuppressive and might contribute to better donor cell engraftment after RIC-HSCT, the rate of engraftment failure after RIC-CBT was still higher than that after RIC-BMT (not significant); however, patients who had experienced graft failure were successfully rescued with a second HSCT. Unrelated cord blood can be an alternative source for RIC-HSCT if a patient has no family donor.