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

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

doi:10.1080/08880018.2022.2075501

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

Pediatr Hematol Oncol. 2023 Mar;40(2):108-116. doi: 10.1080/08880018.2022.2075501. Epub 2022 May 24.

Authors

Thirachit Chotsampancharoen¹, Shevachut Chavananon¹, Pornpun Sripornsawan¹, Natsaruth Songthawee¹, Edward B McNeil²

Affiliations

¹ Faculty of Medicine, Department of Pediatrics, Prince of Songkla University, Hat Yai, Thailand.
² Faculty of Medicine, Epidemiology Unit, Prince of Songkla University, Hat Yai, Thailand.

PMID: 35608385
DOI: 10.1080/08880018.2022.2075501

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.Abbreviations.

Keywords: Acute myeloid leukemia; Down syndrome; transient abnormal myelopoiesis.

MeSH terms

Child, Preschool
Down Syndrome* / complications
Down Syndrome* / epidemiology
Follow-Up Studies
Humans
Infant
Leukemoid Reaction*
Thailand / epidemiology

Supplementary concepts

Myeloproliferative Syndrome, Transient