CAJ | 학술논문

目的提高对原发脾脏NK/T细胞淋巴瘤的认识.方法对 1 例合并EB病毒感染的原发脾脏NK/T细胞淋巴瘤患者的临床特征、病理组织学特点、治疗与预后进行分析,并复习相关文献.结果患者男,40 岁,因"脾大6 年,增长迅速伴发热7 个月"入我院,外周血EBV-DNA复制高达5.2×109 copies/ml,无浅表及腹腔淋巴结、肺、肝累及,骨髓象可见6%的异常淋巴细胞,行脾切除术后明确诊断为脾脏NK/T细胞淋巴瘤.术后1个月肝、肺浸润合并噬血细胞综合征、弥散性血管内凝血、消化道出血、呼吸衰竭,给予地塞米松15 mg/d+依托泊苷50 mg/d+更昔洛韦500 mg/d,连续10 d,外周血EBV-DNA进行性下降、肝肺病灶减少,达部分缓解.结论原发脾脏NK/T细胞淋巴瘤罕见,以脾脏进行性增大伴发热为主要表现,病情呈高度侵袭性,短期内出现噬血细胞综合征、多脏器衰竭,病程凶险,预后差.高EB病毒复制与疾病发生、发展关系密切.依托泊苷联合地塞米松及更昔洛韦作为清除EB病毒、缓解原发脾脏NK/T细胞淋巴瘤合并噬血细胞综合征的方法,近期效果显著.但缓解后仍需积极化疗.
목적 제고대원발비장NK/T세포림파류적인식.방법 대 1 례합병EB병독감염적원발비장NK/T세포림파류환자적림상특정、병리조직학특점、치료여예후진행분석,병복습상관문헌.결과 환자남,40 세,인"비대6 년,증장신속반발열7 개월"입아원,외주혈EBV-DNA복제고체5.2×109 copies/ml,무천표급복강림파결、폐、간루급,골수상가견6%적이상림파세포,행비절제술후명학진단위비장NK/T세포림파류.술후1개월간、폐침윤합병서혈세포종합정、미산성혈관내응혈、소화도출혈、호흡쇠갈,급여지새미송15 mg/d+의탁박감50 mg/d+경석락위500 mg/d,련속10 d,외주혈EBV-DNA진행성하강、간폐병조감소,체부분완해.결론원발비장NK/T세포림파류한견,이비장진행성증대반발열위주요표현,병정정고도침습성,단기내출현서혈세포종합정、다장기쇠갈,병정흉험,예후차.고EB병독복제여질병발생、발전관계밀절.의탁박감연합지새미송급경석락위작위청제EB병독、완해원발비장NK/T세포림파류합병서혈세포종합정적방법,근기효과현저.단완해후잉수적겁화료.
Objective To improve the knowledge in primary splenic NK/T cell lymphoma. Methods The clinical features, histopathologic characteristics, treatment and prognosis of 1 patient with primary splenic NK/T cell lymphoma with EB virus infected was analyzed, and the related literatures were reviewed.Results A 40-year-old man was admitted to Shanxi Dayi Hospital with a 6-year history of splenomegaly, and his splenic grown rapidly with fever for 7 months, with a highly EBV-DNA copy in his peripheral blood reached 5.2×109 copies/ml, without superficial and enterocoelia lymphadenopathy, without involvement in lungs and liver. Bone marrow smear found 6% atypical lymphoma cells. He was definitely diagnosed as splenic NK/T cell lymphoma by splenectomy. 1 month after the splenectomy, the patient was detected concrete masses in his lungs and liver by a computed tomography (CT), and complicated with hemophagocytic syndrome, disseminated intravascular coagulation, hemorrhage of digestive tract and respiratory failure. The patient received an active supporting therapy and a combined therapy of dexamethasone 15 mg/d+etoposide 50 mg/d+ganciclovir 500 mg/d for 10 days and had been partial remission rapidly. The EBV-DNA copy was progressively decreased also.Conclusions Primary splenic NK/T cell lymphoma is rare, which is characterized by progressively enlarging of spleen with fever, highly invasive, hemophagocytic syndrome, multiple organ failure, poor prognosis. High EB viral copy has close relationship with disease's occurrence and development. Etoposide combined with dexamethasone and ganciclovir had been proved play a significant role in eliminating EB virus and relieving primary splenic NK/T cell lymphoma complicated with hemophagocytic syndrome. However, active chemotherapy is still necessary for patients received remission.