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2例

Two Cases of Hydroa Vacciniforme-like Epstein-Barr Virus-associated Lymphoproliferative Disorder

目的根据2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者的临床表现、治疗及转归,探讨该病的诊断和治疗。方法分析2例患者的临床资料、实验室检查、治疗及转归。结果 2例均为幼年发病,皮损开始出现在暴露部位,反复发作,数月或数年后进展至非暴露部位且伴有发热等全身症状,严重者出现肝功能异常及嗜血现象。皮损组织病理示真皮内致密淋巴样细胞浸润至脂肪层,侵犯血管,可见部分中等大小不典型异形淋巴细胞;免疫组化示浸润细胞以CD4+T细胞为主;皮损中EB病毒(Epstein-Barrvirus,EBV)原位杂交阳性,血清中EBV抗体阳性。结论该病与EB病毒感染有关,糖皮质激素联合静脉丙种球蛋白即可控制症状,尚无必要按"淋巴瘤"治疗,但需长期随访。

Objective To study the diagnosis standard,therapy and prognosis of Hydroa vaeciniforme-like Epstein-Barr virus-associated lymphoproliferative disorder in two cases. Methods The clinical data, laboratory examina- tions of two cases of Hydroa vacciniforme-like Epstein-BaiT virus-associated lymphoproliferative disorder were analyzed. Results All patients suffered from the condition in childhood. Their impressive cutaneous rash characterized by edema, blisters, ulcers, crusts, and scars, resembling Hydroa vacciniforme, were seen mainly in light-exposed and non-exposed areas. They had fever and lymphadenopathy. One of the two pa- tients was found to have liver damage and hemophagocytic syndrome. The pathology of skin lesion showed that it consisted of dense lymphomatous T cell infiltration into the epidermis, dermis and subcutis with variable ex- ocytosis and angiocentricity. Immunohistochemical staining showed that most of the infiltrating lymphoid cells were positive for CD4. The EBV genomes were found within tissue from both two skin biopsies and peripheral blood cells. Conclusion An effective therapy is needed to eradicate chronic EBV infection in order to avert potentially fatal outcomes. The patients may be remitted in the treatment with Hormone and IVIG. But, it is too early to treat them as ＂lymphoma＂ before enough evidence are collected. Both of the patients require long time follow-up in case of fatal outcomes.