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来自一个发展中国家的儿童高白细胞急性白血病的临床特征与短期预后

Clinical Profile and Short-Term Outcome of Pediatric Hyperleukocytic Acute Leukemia from a Developing Country.

作者信息

Baqari Syed Ali Shazif, Haque Anwarul, Ashraf Muhammad Shamvil, Alam Muhammad Matloob, Fadoo Zehra

机构信息

Department of Peadiatric and Child Health, The Aga Khan University Hospital, Karachi.

Department of Peadiatric, Children Cancer Hospital, Karachi.

出版信息

J Coll Physicians Surg Pak. 2017 Jul;27(7):450-454.

PMID:28818172
Abstract

This study was conducted to determine the frequency, clinical profile, and short-term outcome of children with hyperleukocytosis at two pediatric oncology centers in Karachi. Of a total 1,045 patients, 13.97% (n=146) patients had hyperleukocytosis. Majority (61.7%, n=90) were under 10 years of age and 76% (n=146) were male. The symptom duration before diagnosis was more than 30 days in 49.3% (n=72). The median WBC count was 181 x109/L(IQR=130.45298.3) and extreme hyperleukocytosis (>200 x109/L) was observed in 44.5% (n=65) patients. Majority (94.5%, n=138) of patients were diagnosed with acute lymphoblastic leukemia. One or more complications developed in 78% (n=114) of cases. Clinical and laboratory tumor lysis syndrome (TLS) was observed in 17.1% (n=25) and 39% (n=57) patients, respectively. Pulmonary and neurological complications related to leukostasis were noted in 9.5% (n=14) and 27.3% (n=40) of cases, respectively. Infectious complications occurred in 23.2% (n=34) patients. The case-specific mortality was 20.5% (n=30). No mortality was related to early complications of hyperleukocytosis.

摘要

本研究旨在确定卡拉奇两家儿科肿瘤中心高白细胞血症患儿的发生率、临床特征和短期预后。在总共1045例患者中,13.97%(n = 146)的患者患有高白细胞血症。大多数(61.7%,n = 90)患者年龄在10岁以下,76%(n = 146)为男性。49.3%(n = 72)的患者诊断前症状持续时间超过30天。白细胞计数中位数为181×10⁹/L(IQR = 130.45 - 298.3),44.5%(n = 65)的患者观察到极高白细胞血症(>200×10⁹/L)。大多数(94.5%,n = 138)患者被诊断为急性淋巴细胞白血病。78%(n = 114)的病例出现一种或多种并发症。分别有17.1%(n = 25)和39%(n = 57)的患者观察到临床和实验室肿瘤溶解综合征(TLS)。分别有9.5%(n = 14)和27.3%(n = 40)的病例出现与白细胞淤滞相关的肺部和神经系统并发症。23.2%(n = 34)的患者发生感染性并发症。病例特异性死亡率为20.5%(n = 30)。没有死亡与高白细胞血症的早期并发症相关。

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