Common blood test indices for predicting transient abnormal myelopoiesis-related mortality in infants with Down syndrome

Published: 14 March 2023| Version 2 | DOI: 10.17632/zm35gr45x3.2
Hideyuki Hawaka, Tomoyuki Shimokaze, Tomoko Yokosuka, Katsuaki Toyoshima, Tomoko Saito, Hiroaki Goto


Information on the prediction of transient abnormal myelopoiesis (TAM)-related mortality at the time of TAM diagnosis is important. Therefore, in this study, we aimed to identify a quantitative index that can be used to predict mortality at the time of diagnosis. In this single-center retrospective review, we analyzed infants with TAM associated with Down syndrome who were admitted to the neonatal intensive care unit in Kanagawa Children’s Medical Center from 1992 to 2021. TAM-related death was defined as death of liver failure or disseminated intravascular coagulation within 6 months of birth. Liver failure was defined as a prothrombin time–international normalized ratio of ≥2.0 under vitamin K administration with biochemical evidence of liver damage according to a report by Squires et al. Intestinal failure-associated liver disease and viral hepatitis were excluded as causes of TAM-related death. The diagnostic criterion for disseminated intravascular coagulation was a score of ≥5 on the International Society on Thrombosis and Haemostasis scoring system. We collected the following data: gestational age, birth weight, sex, Apgar score, congenital heart disease, congenital gastrointestinal disease, tracheal intubation at birth, hydrops, systemic edema, bleeding symptoms, white blood cell count, blast percentage, hemoglobin concentration, platelet count, serum aspartate aminotransferase and alanine aminotransferase concentrations, lactate dehydrogenase concentration, direct bilirubin concentration, albumin concentration, prothrombin time–international normalized ratio, D-dimer concentration at the time of diagnosis, hyaluronic acid concentration, low-dose cytarabine administration, glucocorticoid administration, red blood cell transfusion, fresh frozen plasma transfusion, and platelet transfusion after the diagnosis. Among an initial total of 462 infants with Down syndrome admitted to our hospital from 1992 to 2021, 51 infants were diagnosed with TAM. Eight infants were excluded, and the remaining 43 infants (12 with TAM-related death and 31 survivors) were included in the analysis. In this study, we showed that a serum albumin concentration of <28 g/L at diagnosis was a simple, sensitive, and specific predictor of TAM-related mortality in infants with TAM.



Leukemia in Children, Down Syndrome, Liver Failure, Neonatal Hematology