血管免疫母细胞性T细胞淋巴瘤并发EB病毒阳性弥漫大B细胞淋巴瘤二例并文献复习

Angioimmunoblastic T-cell lymphoma complicated with Epstein-Barr virus-positive diffuse large B-cell lymphoma:report of two cases and review of literature

目的探讨血管免疫母细胞性T细胞淋巴瘤(AITL)并发EB病毒(EBV)阳性弥漫大B细胞淋巴瘤(DLBCL)患者的临床病理特征、治疗及预后。方法回顾性分析解放军总医院第五医学中心2例AITL并发EBV阳性DLBCL患者的临床资料,并进行文献复习。结果例1为混合淋巴瘤(CL)患者,以低热伴全身浅表淋巴结肿大起病,右侧腋窝肿物活组织检查示AITL并发EBV阳性DLBCL,予以8个周期化疗后达不确定的完全缓解;后续应用西达本胺维持治疗,仍生存中。例2为不一致性淋巴瘤(DL)患者,以皮下结节起病,后出现浅表淋巴结进行性肿大;皮下结节病理检查诊断为DLBCL,右腹股沟淋巴结病理检查诊断为AITL;接受7个周期化疗,因合并噬血细胞综合征而死亡。结论AITL合并EBV阳性DLBCL罕见,临床症状主要以AITL的表现为主,存在T细胞及B细胞免疫表型特征,预后差,治疗方案主要依据预后较差的淋巴瘤进行选择。

Objective To investigate the clinicopathological features,treatment and prognosis of patients with angioimmunoblastic T-cell lymphoma(AILT)complicated with Epstein-Barr virus(EBV)-positive diffuse large B-cell lymphoma(DLBCL).Methods The clinical data of 2 cases of AILT with EBV-positive DLBCL in the Fifth Medical Center of PLA General Hospital were retrospectively analyzed,and the literature was reviewed.Results One case of complex lymphoma(CL)developed from low fever with systemic superficial lymphadenopathy,the right axillary tumor biopsy showed that AILT complicated with EBV-positive DLBCL.After 8 cycles of chemotherapy,the patient reached unconfirmed complete remission,then received maintenance treatment with chidamide and survived until the deadline.Another case of discordant lymphoma began with subcutaneous nodules and then developed progressive enlargement of superficial lymph nodes.The pathological examination of subcutaneous nodule diagnosed as DLBCL,and the pathological examination of right inguinal lymph node diagnosed as AITL.After 7 cycles of chemotherapy,the patient died of hemophagocytic syndrome.Conclusions AILT with EBV-positive DLBCL is rare.The main clinical symptom is AITL and the immunophenotypic characteristic indicates the presence of T cells and B cells.At the same time,the prognosis is poor,and the treatment strategy mainly depends on the lymphoma type with unfavorable prognosis.