Abstract

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.

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摘要

短暂性异常骨髓细胞生成 (TAM) 是一种发生在唐氏综合症 (DS) 婴儿中的独特疾病，大多数患者可自发缓解。本研究旨在评估泰国三级中心 TAM 的发生率和结果。我们审查了 1993 年 6 月至 2019 年 10 月期间诊断出的 997 名 DS 患者的记录。在这 997 名 DS 患者中，有 32 名被诊断出患有 TAM。TAM 的发生率为 3.2%，总生存率为 87.5%。共有 2/28 名幸存者 (7.1%) 随后分别在 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产妇多胎、败血症、皮肤出血、皮下结节、高白细胞计数、低血红蛋白和升高的 AST 水平。

缩略语