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目的分析种痘水疱病样淋巴瘤3例患儿特点,探讨该病的诊断与治疗,提高临床医生对其认识。方法总结2007年11月至2011年10月中南大学湘雅医院诊治的3例种痘水疱病样淋巴瘤患儿的临床表现、实验室检查、诊治经过,并结合相关文献进行分析。结果 (1)主要临床表现为多形性皮疹、间歇性发热、肝脾淋巴结肿大及凹陷性水肿。(2)实验室检查:3例患儿血象分类均以淋巴细胞为主,2例免疫组化示CD45RO(+)、CD56(+)、TIA-1(+)、Ki67(>30%),1例免疫组化示CD3ε(+)、CD56(+)、TIA-1(+)、Ki67(>30%)、EBER(原位杂交)(+)。皮肤活检:2例示皮肤种痘水疱病样T细胞淋巴瘤,1例示皮肤T细胞非霍奇金恶性淋巴瘤,倾向外周T细胞性。(3)治疗及预后:病例1放弃治疗;病例2丙种球蛋白及激素治疗2周后皮疹明显改善,予以化疗(柔红霉素+门冬酰胺酶+地塞米松+长春新碱),骨髓抑制期后因消化道出血死亡;病例3因侵犯多器官致全身衰竭死亡。结论种痘水疱病样淋巴瘤确诊主要依据皮损部位组织病理活检及免疫组化,发病与EB病毒感染有关。不同疗法的治疗反应存在个体差异,应综合患儿对治疗的反应及其他因素,采取个性化治疗。本病病情进展迅速,恶性程度高,预后差。
Objective To analyze the characteristics of 3 kinds of children with acne blister type lymphoma, discuss the diagnosis and treatment of this disease and improve the understanding of clinicians. Methods The clinical manifestations, laboratory tests, diagnosis and treatment of 3 children with acne blister lymphoma diagnosed and treated by Xiangya Hospital of Central South University from November 2007 to October 2011 were summarized and analyzed with related literatures. Results (1) The main clinical manifestations of pleomorphic rash, intermittent fever, hepatosplenomegaly and pitting edema. (2) Laboratory examination: Blood samples were collected from 3 patients with lymphocytic leukemia. Immunohistochemistry showed CD45RO (+), CD56 (+), TIA-1 (+) and Ki67 (> 30% One case of immunohistochemistry showed CD3ε (+), CD56 (+), TIA-1 (+), Ki67 (> 30%) and EBER (+). Skin biopsy: 2 cases of skin acne blisters like T-cell lymphoma, 1 case of skin T cell non-Hodgkin’s lymphoma, the tendency of peripheral T cell. (3) treatment and prognosis: Case 1 to give up treatment; case 2 gamma globulin and hormone treatment 2 weeks after the rash was significantly improved, to chemotherapy (daunorubicin + asparaginase + dexamethasone + vincristine), bone marrow After the suppression of death due to gastrointestinal bleeding; Case 3 due to multiple organ damage caused by systemic failure. Conclusion The diagnosis of acne blister-like lymphoma is mainly based on the histopathological biopsy and immunohistochemistry of the lesions. The incidence is related to Epstein-Barr virus infection. Different treatment response to the existence of individual differences, should be integrated in children’s response to treatment and other factors, to take personalized treatment. The rapid progression of the disease condition, high grade, poor prognosis.