唐氏综合征的短暂性骨髓异常:泰国单一三级中心的长期随访经验。

IF 1.2 4区医学 Q4 HEMATOLOGY Pediatric Hematology and Oncology Pub Date : 2023-03-01 DOI:10.1080/08880018.2022.2075501

Thirachit Chotsampancharoen, Shevachut Chavananon, Pornpun Sripornsawan, Natsaruth Songthawee, Edward B McNeil

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引用次数: 2

摘要

短暂性骨髓增生异常(TAM)是一种发生在唐氏综合征(DS)婴儿中的独特疾病，大多数患者会自发缓解。本研究旨在评估泰国某三级医疗中心TAM的发病率和预后。我们回顾了1993年6月至2019年10月期间诊断的997名DS患者的记录。在997例DS患者中，32例被诊断为TAM。TAM的发生率为3.2%，总生存率为87.5%。共有2/28的存活患者(7.1%)分别在2.1岁和4.5岁时发生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、白细胞计数高、血红蛋白低和AST水平升高。

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Transient abnormal myelopoiesis in Down syndrome: Experience of long term follow up from a single tertiary center in Thailand.

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.

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来源期刊

Pediatric Hematology and Oncology 医学-小儿科

CiteScore

2.60

自引率

5.90%

发文量

审稿时长

6-12 weeks

期刊介绍： PHO: Pediatric Hematology and Oncology covers all aspects of research and patient management within the area of blood disorders and malignant diseases of childhood. Our goal is to make PHO: Pediatric Hematology and Oncology the premier journal for the international community of clinicians and scientists who together aim to define optimal therapeutic strategies for children and young adults with cancer and blood disorders. The journal supports articles that address research in diverse clinical settings, exceptional case studies/series that add novel insights into pathogenesis and/or clinical care, and reviews highlighting discoveries and challenges emerging from consortia and conferences. Clinical studies as well as basic and translational research reports regarding cancer pathogenesis, genetics, molecular diagnostics, pharmacology, stem cells, molecular targeting, cellular and immune therapies and transplantation are of interest. Papers with a focus on supportive care, late effects and on related ethical, legal, psychological, social, cultural, or historical aspects of these fields are also appreciated. Reviews on important developments in the field are welcome. Articles from scientists and clinicians across the international community of Pediatric Hematology and Oncology are considered for publication. The journal is not dependent on or connected with any organization or society. All submissions undergo rigorous peer review prior to publication. Our Editorial Board includes experts in Pediatric Hematology and Oncology representing a wide range of academic and geographic diversity.