【摘要】 目的:探讨血液病患者合并鼻眶脑毛霉菌感染的临床特征、诊断、治疗及预后。方法:回顾性分析多中心收治的15例血液病合并鼻眶脑毛霉菌感染患者的临床资料，对其人口学特征、原发病、临床表现、影像学特征、病原学结果、治疗方案及转归进行分析。结果:15例患者中，男11例，女4例，中位年龄59(19～77)岁。原发病包括再生障碍性贫血6例，急性髓系白血病4例，骨髓增生异常肿瘤3例，原发中枢淋巴瘤1例，T淋巴母细胞淋巴瘤1例。病原学以根霉属最常见(7例，其中小孢根霉5例)。临床主要表现面部肿胀疼痛(46.67%)及眶周肿胀或眼睑下垂(46.67%)。12例患者接受两性霉素B(包括脂质体及胆固醇硫酸酯复合物)单药或联合药物治疗，3例接受泊沙康唑或艾沙康唑单药治疗。死亡9例(60%)，且均在确诊后3周内死亡。6例(40%)治愈，其中2例接受了联合手术治疗。结论:血液病合并鼻眶脑毛霉菌感染病情凶险、进展迅速、病死率高。治疗关键在于早期应用抗毛霉菌药物，并积极联合手术清创，有望改善患者预后。更多还原

【Abstract】 Objective: To investigate the clinical characteristics,diagnosis,treatment,and prognosis of hematologic diseases complicated by rhino-orbito-cerebral mucormycosis. Methods: A multicenter retrospective analysis was conducted on clinical data from 15 patients with hematologic diseases complicated by rhino-orbito-cerebral mucormycosis. Demographic characteristics,primary diseases,clinical manifestations,imaging features,etiological results,treatment regimens,and outcomes were analyzed. Results: Among the 15 patients,there were 11 males and 4 females,with a median age of 59 years( range,19-77 years). The primary diseases included aplastic anemia( 6 cases),acute myeloid leukemia( 4 cases),myelodysplastic neoplasms( 3 cases),primary central nervous system lymphoma( 1 case),and T-lymphoblastic lymphoma( 1 case). The most common pathogen was Rhizopus species( 7 cases,including Rhizopus microsporus in 5 cases). The main clinical manifestations were facial swelling and pain( 46. 67%) and periorbital swelling or ptosis( 46. 67%). Twelve patients received amphotericin B( including liposomal and cholesterol sulfate complex formulations) as monotherapy or combination therapy,3 patients received isavuconazole or posaconazole monotherapy. Nine patients( 60%) died,all within 3 weeks after diagnosis. Six patients( 40%) achieved cure,of whom 2 received combined surgical treatment. Conclusion:Hematologic diseases complicated by rhino-orbito-cerebral mucormycosis are associated with a fulminant clinical course,rapid progression,and high mortality. The key to improving prognosis lies in early administration of antifungal agents active against mucormycetes and active combined surgical debridement.更多还原