Arya L S, Narain Sunil, Tomar Sanjay, Thavaraj Vasantha, Dawar R, Bhargawa M
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi.
Indian J Pediatr. 2002 Apr;69(4):293-7. doi: 10.1007/BF02723212.
In order to study the clinical profile of children presenting with superior vena cava syndrome (SVCS), case records of all children presenting with SVCS over a 10-year period were retrospectively analyzed.
Twenty one children (20 males and 1 female) with a median age of 10.0 years (range 5.0 to 12.0 years) were detected to have an underlying hematological malignancy. T-cell acute lymphoblastic leukemia accounted for a major share (57%) in the underlying etiology; 33% of the subjects had non-Hodgkin's lymphoma (T-cell lymphoblastic lymphoma).
No Patient required radiotherapy or urgent thoracotomy. Among survivors (n=11), who were alive and well at the time of writing this report, the median follow-up was 6.5 years, (range 0.5-9.6 years). T-cell ALL and lymphoblastic lymphoma are common underlying causes of SVCS in Indian children.
The value of diagnostic interventions performed under local anaesthesia and prebiopsy corticosteroids usage was found to be reassuring since long-term survival without disease is achievable.
为研究表现为上腔静脉综合征(SVCS)的儿童的临床特征,对10年间所有表现为SVCS的儿童的病例记录进行回顾性分析。
检测到21名儿童(20名男性和1名女性)患有潜在血液系统恶性肿瘤,中位年龄为10.0岁(范围5.0至12.0岁)。T细胞急性淋巴细胞白血病在潜在病因中占主要比例(57%);33%的受试者患有非霍奇金淋巴瘤(T细胞淋巴母细胞淋巴瘤)。
无患者需要放疗或紧急开胸手术。在撰写本报告时仍存活且状况良好的幸存者(n = 11)中,中位随访时间为6.5年(范围0.5 - 9.6年)。T细胞急性淋巴细胞白血病和淋巴母细胞淋巴瘤是印度儿童SVCS的常见潜在病因。
发现局部麻醉下进行诊断性干预和活检前使用皮质类固醇的价值令人放心,因为可以实现无病长期生存。