Stefan Daniela Cristina, Van Toorn Ronald, Andronikou Savvas
Department of Pediatrics and Child Health, Faculty of Health Sciences, Stellenbosch University and Tygerberg Children's Hospital, Tygerberg, Cape Town, South Africa.
J Pediatr Hematol Oncol. 2009 Apr;31(4):252-5. doi: 10.1097/MPH.0b013e31818c918a.
We describe a newly diagnosed HIV-infected child, without prior history of AIDS-defining disease, who presented with Burkitt lymphoma-related cauda equina syndrome that rapidly progressed to a flaccid paraplegia. Diagnosis was confirmed on biopsy and magnetic resonance imaging of the spine showed multiple epidural masses with involvement of several vertebral bodies, cord edema and compression of the cord and cauda equina. The child's immune status was relatively preserved and Epstein-Barr serology proved negative. Chemotherapy (LMB 89 modified protocol) was initiated immediately after histopathologic confirmation, together with highly active antiretroviral therapy. A follow-up magnetic resonance imaging 6 weeks later showed segmental cord atrophy at the site of previous edema despite complete resolution of all the epidural masses. Unfortunately, the child's neurologic state did not improve beyond the recovery of sphincter control and lower limb sensation. The patient is the first reported case of Burkitt lymphoma-related spinal cord compression as an initial AIDS-defining illness, in a 10-year-old child. The outcome of the case highlights the importance of early diagnosis and prompt treatment of this aggressive tumor to avoid permanent neurologic deficits.
我们描述了一名新诊断出感染艾滋病毒的儿童,此前无艾滋病界定疾病史,该儿童出现了与伯基特淋巴瘤相关的马尾综合征,并迅速发展为弛缓性截瘫。活检确诊,脊柱磁共振成像显示多个硬膜外肿块,累及多个椎体,脊髓水肿,脊髓和马尾受压。该儿童的免疫状态相对保留,爱泼斯坦-巴尔血清学检查结果为阴性。组织病理学确诊后立即开始化疗(LMB 89改良方案),同时进行高效抗逆转录病毒治疗。6周后的随访磁共振成像显示,尽管所有硬膜外肿块已完全消退,但先前水肿部位出现节段性脊髓萎缩。不幸的是,该儿童的神经状态除括约肌控制和下肢感觉恢复外未得到改善。该患者是首例报道的以伯基特淋巴瘤相关脊髓压迫作为初始艾滋病界定疾病的10岁儿童病例。该病例的结果凸显了早期诊断和及时治疗这种侵袭性肿瘤以避免永久性神经功能缺损的重要性。