血液病合并毛霉菌病七例报道并文献复习

Mucormycosis in patients with hematological diseases:seven cases reports and literature review

目的探讨血液病合并毛霉菌病患者的临床特征、诊治与转归,提高对该病的认识。方法回顾性分析2012年9月至2018年9月7例血液病合并毛霉菌病患者的临床资料,分析其临床特征、治疗过程和转归。结果7例血液病合并毛霉菌病患者中,男4例,女3例,中位年龄36(19~79)岁。基础疾病:急性髓系白血病2例,急性B淋巴细胞白血病、外周T细胞淋巴瘤、慢性髓性白血病急髓变、骨髓增殖性肿瘤及重型再生障碍性贫血(移植后)各1例。毛霉菌病临床类型:4例为肺型,1例为鼻眶脑型,1例为皮肤型,1例为播散型。7例患者均通过活检组织病理确诊。治疗药物为两性霉素B、两性霉素B脂质体及泊沙康唑。手术治疗4例,其中3例术中彻底清除病变组织,1例仅行局限性清除。治愈2例,好转1例,死亡4例。结论血液病合并毛霉菌病患者临床表现及影像学表现多样,死亡率高,确诊主要依赖于组织病理学。早期诊断、控制基础疾病、改善免疫抑制状态、尽早行有效的抗真菌药物治疗及彻底的手术清创治疗是提高血液病合并毛霉菌病患者生存率的关键。

Objective To explore the clinical characteristics,diagnosis,treatment and prognosis of patients with hematological diseases that complicated by mucormycosis,and to improve the understanding and clinical diagnosis and treatment of the disease.Methods The clinical data of 7 patients suffering from mucormycosis during September 2012 and September 2018 were retrospectively analyzed,and their clinical characteristics,treatment process and prognosis were analyzed.Results Of 7 patients,there were 4 males and 3 females,with a median age of 36(19-79)years old.Two patients were diagnosed as acute myeloid leukemia as the underlying disease,the other 5 patients suffered from acute B lymphoblastic leukemia,peripheral T cell lymphoma,chronic myelocytic leukemia in blastic phase,myeloproliferative neoplasm and severe aplastic anemia after transplantation,respectively.Among them,disease types of mucormycosis were pulmonary in 4 patients,rhino-orbital-cerebral in 1 patient,cutaneous in 1 patient and disseminated in 1 patient.All the cases were confirmed by biopsy histopathology.The treatment drugs were amphotericin B or liposomal amphotericin B,and posaconazole.Surgical treatment was performed in 4 patients,3 out of 4 achieved radical debridement,and the other one had local debridement.Two patients were cured,1 patient was improved and 4 patients died.Conclusions The clinical manifestation and image feature of mucormycosis in patients with hematological diseases were diverse,and the mortality rate is high,diagnosis mainly depends on histopathology.Early diagnosis,control of underlying disease,improvement of immunosuppressive status,timely effective antifungal therapy and radical surgical debridement are the key points for improving the survival rate of patients with hematological diseases complicated by mucormycosis.