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目的:报道以EB病毒相关噬血细胞淋巴组织细胞增生症(EBV-HLH)为最初起病症状的结外鼻型NK/T细胞淋巴瘤1例并进行了文献综述。方法:总结分析以EBV-HLH为最初起病症状的1例成人结外(鼻型)NK/T细胞淋巴瘤的临床资料。详细采集患者病史、体格检查、外周血和骨髓化验、PET-CT影像学及组织病理学等检查资料总结分析,并同时进行文献复习。结果:患者,女,27岁,因“发热伴咽痛、咳嗽和关节酸痛1月”入院。入院检查发现全血细胞减少、发热伴肝脾肿大,结合EBV阳性支持EBV-HLH,故予免疫抑制为主的治疗方案,此后患者血常规一度好转。1月后出现带状疱疹,并进行性出现鼻塞。下鼻甲活检病理示浸润细胞CD56+/-,TIA1+,GB+,PF+,CD3+,CD43+,L26-,PAX5-,CD21-,CD30-,KP1-,Ki-67+(约60%),EREB+,诊断为结外鼻型NK/T细胞性淋巴瘤。骨髓像同时找到噬血组织细胞。患者接受了联合VP16的化疗和鼻部放疗,但最终疾病未能控制而死亡。从最初诊断为HLH至最后死亡总共生存6个月。结论:本病例演示了从“EBV-HLH进展至鼻区NK/T细胞淋巴瘤”整个过程,探讨了EBV感染,HLH和NK/T淋巴瘤在发病机制上的相关性。结合文献,伴HLH的淋巴瘤预后差,而HLH出现在淋巴瘤之前较罕见,并且预后更差。目前尚缺乏标准的有效治疗方法。有报道对于高危患者尽早使用含VP16免疫化疗方案和异基因造血干细胞移植可改善患者生存,但仍需积累更多的成功治疗经验指导临床实践,以提高患者的生存率。
OBJECTIVE: To report a case of extranodal nasal type NK / T cell lymphoma with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) as the initial onset symptom and literature review. Methods: The clinical data of 1 adult extranodal (nasal) NK / T cell lymphoma with EBV-HLH as initial symptom onset were summarized. Detailed collection of patient history, physical examination, peripheral blood and bone marrow tests, PET-CT imaging and histopathology examination data summary analysis, and at the same time review of the literature. Results: The patient, female, 27 years old, was admitted to hospital because of “fever with sore throat, cough and joint pain January”. Admission examination found pancytopenia, fever with hepatosplenomegaly, combined with EBV-positive support for EBV-HLH, so immunosuppressive-based treatment program, since the blood was once the patient improved. Shingles appear after 1 month and progressive nasal congestion occurs. The inferior turbinate biopsy showed infiltration of CD56 +/-, TIA1 +, GB +, PF +, CD3 +, CD43 +, L26-, PAX5-, CD21-, CD30-, KP1-, Ki- 67+ (about 60%), EREB + Extranodal nasal type NK / T cell lymphoma. Bone marrow also found hemophagocytic cells. Patients underwent chemotherapy and nasal radiotherapy combined with VP16, but eventually died of uncontrollable disease. A total of 6 months of survival from initial diagnosis of HLH to final death. CONCLUSIONS: This case demonstrates the progression from EBV-HLH to nasal NK / T-cell lymphoma and explores the pathogenesis of EBV infection, HLH and NK / T lymphoma. In combination with the literature, lymphoma with HLH has a poor prognosis, whereas HLH appears rare before lymphoma and has a worse prognosis. Currently there is a lack of standard and effective treatment. It has been reported for high-risk patients as soon as possible with VP16 immunotherapy and chemotherapy and allogeneic hematopoietic stem cell transplantation can improve patient survival, but still need to accumulate more successful treatment experience to guide clinical practice to improve patient survival.