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在毛霉菌病活动期行造血干细胞移植成功治疗重型再生障碍性贫血

Successful Treatment of Severe Aplastic Anemia with Hematopoietic Stem Cell Transplantation in the Setting of Active Mucormycosis.

作者信息

Zhumatayev Suleimen, Celen Safiye Suna, Kara Manolya, Selcuk Ayse Adin, Bozkurt Betül, Demir Mustafa Kemal, Yalcın Koray

机构信息

Department of Pediatric Hematology, Bahçeşehir University School of Medicine, Istanbul, Turkey.

Department of Pediatric Infectious Diseases, Yeditepe University School of Medicine, Istanbul, Turkey.

出版信息

Indian J Otolaryngol Head Neck Surg. 2024 Aug;76(4):3552-3555. doi: 10.1007/s12070-024-04610-1. Epub 2024 Apr 4.

Abstract

Severe aplastic anemia (SAA) is a life-threatening hematological disease characterized by the suppression of the bone marrow. Patients with SAA are predisposed to recurrent bacterial infections and invasive fungal infections (IFI) due to profound and persistent neutropenia. Mucorales are the second most common cause of IFI encountered in SAA. Here we present a pediatric case of SAA with active mucormycosis infection of the paranasal sinuses. In the first step, surgical debridement was performed and combined antifungal therapy (liposomal amphotericin B, posaconazole, caspofungin) was started. Due to severe neutropenia, daily granulocyte transfusion was added to therapy. Hyperbaric oxygen therapy was applied for wound healing. After all this the patient went under flap surgery. One week after the successful flap procedure, HSCT was performed and he had no complications related to HSCT. The patient was followed in the outpatient clinic for 6 months with posaconazole. Now, he is out of drugs and followed without problems for 15 months after HSCT. Our case confirms that urgent HSCT with multiple therapies (surgical debridement, granulocyte support, combined antifungal therapy, hyperbaric O2) is crucial for saving life in SAA patients with active mucormycosis.

摘要

重型再生障碍性贫血(SAA)是一种危及生命的血液系统疾病,其特征为骨髓抑制。由于严重且持续的中性粒细胞减少,SAA患者易发生反复细菌感染和侵袭性真菌感染(IFI)。毛霉目真菌是SAA患者中IFI的第二大常见病因。本文介绍了1例患有鼻窦活跃性毛霉病感染的儿童SAA病例。第一步进行了手术清创,并开始联合抗真菌治疗(脂质体两性霉素B、泊沙康唑、卡泊芬净)。由于严重中性粒细胞减少,治疗中增加了每日粒细胞输注。采用高压氧疗法促进伤口愈合。在此之后,患者接受了皮瓣手术。皮瓣手术成功一周后,进行了造血干细胞移植(HSCT),且未出现与HSCT相关的并发症。患者在门诊接受泊沙康唑治疗6个月。现在,他已停药,HSCT后15个月随访无异常。我们的病例证实,对于患有活跃性毛霉病的SAA患者,采用多种疗法(手术清创、粒细胞支持、联合抗真菌治疗、高压氧)进行紧急HSCT对挽救生命至关重要。

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Sequence of oral manifestations in rhino-maxillary mucormycosis.鼻上颌毛霉菌病的口腔表现顺序
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Infections in patients with aplastic anemia.再生障碍性贫血患者的感染
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Aplastic anemia.再生障碍性贫血
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Clin Infect Dis. 1993 Jan;16(1):1-7. doi: 10.1093/clinids/16.1.1.
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Laryngoscope. 1980 Apr;90(4):635-48. doi: 10.1288/00005537-198004000-00010.

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