中枢神经系统真菌感染(CNSFI)见于血液系统恶性肿瘤患者,具有很高的发病率和死亡率。因为它们的稀有性,在没有既定治疗方案或指南的儿童中,关于CNSFI的数据有限.
在这项多中心回顾性研究中,51名儿童白血病患者,其中6人接受过骨髓移植,对已证实或可能的CNSFI进行了评估。根据欧洲癌症研究和治疗组织标准,真菌感染被定义为已证实或可能。通过适当的中枢神经系统(CNS)成像或组织样本发现以及脑脊液的阳性微生物学结果来诊断经证实的CNSFI。积极的文化,菌丝的微观证据,半乳甘露聚糖检测的阳性结果被定义为阳性微生物证据。如果没有其他解释条件,可能的CNSFI被定义为适当的CNS影像学发现以及在没有CNS的另一个病灶处已证实或可能的侵袭性真菌感染。数据是通过使用问卷调查表(补充数字内容1,http://链接。www.com/JPHO/A541).
17名患者证实,34例患者有可能的CNSFI。头痛和癫痫发作是最常见的临床表现。从发烧到诊断的中位时间为5天。鉴定的最常见的真菌剂是曲霉属。16名患者接受了单一药物治疗,35例接受联合抗真菌治疗。23例患者进行了手术。22名患者(43%)死亡,CNSFI发作中的29例恢复了20%的神经系统后遗症。
在白血病和难治性/复发性发热患者的鉴别诊断中应考虑CNSFIs,头痛,神经系统的眼部症状,应立即进行放射学-血清学评估。早期诊断和及时管理,医学和外科,对改善临床结果至关重要。
Central nervous system fungal infections (CNSFI) are seen in patients with hematologic malignancies and have high morbidity and mortality. Because of their rarity, there is limited data on CNSFI in children with no established treatment protocols or guidelines.
In this multicenter retrospective
study, 51 pediatric patients with leukemia, 6 of whom had undergone bone marrow transplantation, with proven or probable CNSFI were evaluated. Fungal infections were defined as proven or probable based on European Organisation for Research and Treatment of Cancer criteria. Proven CNSFI was diagnosed by appropriate central nervous system (CNS) imaging or tissue sample findings in combination with positive microbiological results of cerebrospinal fluid. A positive culture, microscopic evidence of hyphae, a positive result of the galactomannan assays are defined as positive microbiological evidence. Probable CNSFI was defined as appropriate CNS imaging findings together with proven or probable invasive fungal infections at another focus without CNS when there is no other explanatory condition. Data was collected by using the questionnaire form (Supplemental Digital Content 1, http://links.lww.com/JPHO/A541 ).
Seventeen patients had proven, 34 patients had probable CNSFI. Headaches and seizures were the most common clinical findings. The median time between the onset of fever and diagnosis was 5 days. The most common fungal agent identified was Aspergillus . Sixteen patients received single-agent, 35 received combination antifungal therapy. Surgery was performed in 23 patients. Twenty-two patients (43%) died, 29 of the CNSFI episodes recovered with a 20% neurological sequelae.
CNSFIs should be considered in the differential diagnosis in patients with leukemia and refractory/recurrent fever, headache, neurologicalocular symptoms, and a radiologic-serological evaluation should be performed immediately. Early diagnosis and prompt management, both medical and surgical, are essential for improving clinical outcomes.