Zorzou Markela-P, Chini Maria, Lioni Athina, Tsekes Georgios, Nitsotolis Thomas, Tierris Ioannis, Panagiotou Nicolaos, Rontogianni Dimitra, Harhalakis Nicolaos, Lazanas Marios
Infectious Diseases Unit, Evangelismos General Hospital of Athens , Greece.
Surgery Department, Evangelismos General Hospital of Athens , Greece.
Hematol Rep. 2016 Dec 22;8(4):6581. doi: 10.4081/hr.2016.6581. eCollection 2016 Nov 2.
Although the connection of [secondary hemophagocytic syndrome (sHS)] with HIV has been well documented, optimal treatment regimen is not well established. This is due not only to the rarity of the syndrome, but also to the heterogeneity of the involved population. Most cases are related to opportunistic infections or malignancies in advanced stage, but many cases are also related to seroconversion, in the primary infection setting. Moreover, in the [antiretroviral treatment (ART)] era, rare cases of ART-related sHS have been reported. In these, often fatal cases, an [immune reconstitution inflammatory syndrome (IRIS)] process is involved, posing a serious challenge to the treating physician. We hereby report a case of successful treatment of an HIV patient with primary effusion lymphoma who experienced sHS shortly after ART onset. Our patient, treated with high dose dexamethasone and gamma globulin, achieved complete remission. This case might hint possible therapeutic insights in the treatment of IRIS-related sHS.
尽管[继发性噬血细胞综合征（sHS）]与HIV的关联已有充分记载，但最佳治疗方案尚未确立。这不仅是因为该综合征罕见，还因为受累人群的异质性。大多数病例与晚期机会性感染或恶性肿瘤有关，但许多病例也与初次感染时的血清转化有关。此外，在[抗逆转录病毒治疗（ART）]时代，已有ART相关sHS的罕见病例报道。在这些往往致命的病例中，涉及[免疫重建炎症综合征（IRIS）]过程，给治疗医生带来了严峻挑战。我们在此报告一例原发性渗出性淋巴瘤的HIV患者在ART开始后不久发生sHS并成功治疗的病例。我们的患者接受了高剂量地塞米松和丙种球蛋白治疗，实现了完全缓解。该病例可能为IRIS相关sHS的治疗提供可能的治疗思路。
