【摘要】 目的:探讨EB病毒感染伴随噬血细胞综合征和霍奇金淋巴瘤的临床特点及转归。方法:骨髓细胞形态用骨髓涂片在光学显微镜下观察,骨髓病理变化用骨髓活检及免疫组织化学方法分析,而淋巴结病理改变用免疫组织化学方法检测。对患者应用ABVD(表柔比星、博来霉素、长春新碱、达卡巴嗪)化学方案治疗。结果:患者发热合并全血细胞减少、铁蛋白和s CD25显著增高,低纤维蛋白原血症,骨髓有噬血现象,EBV-DNA拷贝数增高,符合EB病毒相关噬血细胞综合征,经抗感染治疗效果不佳,按HLH-2004方案治疗,患者恢复体温正常,全血细胞、铁蛋白和纤维蛋白原均恢复正常;左下颌淋巴结肿大,活检病理诊断为经典例型霍奇金淋巴瘤(混合细胞型),给予ABVD(表柔比星、博来霉素、长春新碱、达卡巴嗪)方案化疗1疗程,淋巴结肿大消退,EBV-DNA拷贝数下降。患者噬血细胞综合征得到有效控制,霍奇金淋巴瘤处于完全缓解。结论:EB病毒相关噬血细胞综合征、霍奇金淋巴瘤在临床少见,其长期预后仍有待于进一步探索。更多还原

【Abstract】 Objective: To investigate the clinical characteristics and outcome of parhents with EBV infection conbined with hemophagocytic syndrome and Hodgkin’s lymphoma. Methods: The morphotogy of bone marrow cells was observed by bone marrow smear and light microscopy, the pathologic changes of bone marrow ware analyzed by bone marrow biopsy and immunohistochemistry methord, the pathologic changes of lymphonudes ware detected by immunohistochemical methord, the paticnts were treated with ABVD(epirubicin, bleomycin, vincristine and dacarbazine) chemotherapeutic regimen. Results: Fever complicatid with pancytopenia, obvious increase of ferritin and sCD25, hypofibrinogenemia, hemophogocytic phenomen of bone marrow, increase of EBV-DNA copy number ware observed, which all accorded with the criteria EBV righted hemophagocytic syndrome. The curative efficacy of amtiinfective treatmatnt was poor, After treatment with HLH-2004 regimen, the fever symptome and the laboratory indicaters such as whole blood cells, ferritin and fibrinogen all were recovered to normal levels. Left mandibular lymphadenctasis was confirmed as Hodgkin’s lymphoma(mixed cell type) by pathological examination. The patient achieved complete molecular remission after 1 course chemotherapy with ABVD regimen. The level of EBV-DNA copy number were also decreased. As the reshlt, the patient’s hemophagocytic syndrome had bean effectively controlled,and the Hodgkin’s lymphoma is still in complete remission. Conclusion: Epstein-Barr virus-ratated hemophagocytic syndrome and Hodgkin’s lymphoma are rare, and their long-term prognosis needs to be further explored.更多还原