唐氏综合征中的短暂性髓系增生异常：来自泰国单一三级医疗中心的长期随访经验。
Transient abnormal myelopoiesis in Down syndrome: Experience of long term follow up from a single tertiary center in Thailand.
机构信息
Faculty of Medicine, Department of Pediatrics, Prince of Songkla University, Hat Yai, Thailand.
Faculty of Medicine, Epidemiology Unit, Prince of Songkla University, Hat Yai, Thailand.
出版信息
Pediatr Hematol Oncol. 2023 Mar;40(2):108-116. doi: 10.1080/08880018.2022.2075501. Epub 2022 May 24.
Transient abnormal myelopoiesis (TAM) is a unique disease occurring in Down syndrome (DS) infants from which most patients have spontaneous remission. This study aimed to evaluate the incidence and outcomes of TAM in a tertiary center in Thailand. We reviewed the records of 997 DS patients diagnosed between June 1993 and October 2019. From the 997 DS patients, 32 had been diagnosed with TAM. The incidence of TAM was 3.2% and an overall survival rate of 87.5%. A total of 2/28 who survived (7.1%) subsequently developed AML-DS at the ages of 2.1 and 4.5 years, respectively. The risk factors related with death included maternal multiparity, sepsis, skin bleeding, subcutaneous nodules, high WBC count, low hemoglobin, and elevated AST level.Abbreviations.
暂时性髓系增生异常（TAM）是一种独特的疾病，发生在唐氏综合征（DS）婴儿中，大多数患者会自发缓解。本研究旨在评估泰国一家三级中心 TAM 的发生率和结局。我们回顾了 1993 年 6 月至 2019 年 10 月期间诊断的 997 例 DS 患者的记录。在 997 例 DS 患者中，有 32 例被诊断为 TAM。TAM 的发生率为 3.2%，总生存率为 87.5%。28 例存活者中有 2 例（7.1%）分别在 2.1 岁和 4.5 岁时发展为 AML-DS。与死亡相关的危险因素包括多产妇、败血症、皮肤出血、皮下结节、白细胞计数高、血红蛋白低和 AST 水平升高。
缩写词。
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