Chemokine levels predict progressive liver disease in Down syndrome patients with transient abnormal myelopoiesis

Background Transient abnormal myelopoiesis(TAM) is a neonatal preleukemic syndrome that occurs exclusively in neonates with Down syndrome (DS). Most affected infants spontaneously resolve, although some patients culminate in hepatic failure despite the hematological remission. It is impossible to determine the patients who are at high risk of progressive liver disease and leukemic transformation. The objective is to search for biomarkers predicting the development of hepatic failure in DS infants with TAM. Methods Among 60 newborn infants with DS consecutively admitted to our institutions from 2003 to 2016, 41 infants with or without TAM were enrolled for the study. Twenty-two TAM-patients were classified into “progression group” (n = 7) that required any therapy and “spontaneous resolution group” (n = 15). Serum concentrations of chemokines (CXCL8, CXCL9, CXCL10, CCL2and CCL5) and transforming growth factor (TGF)-β1 were measured at diagnosis of TAM for assessing the outcome of progressive disease. Results Three patients developed leukemia during the study period (median, 1147 days; range, 33–3753). Three died of hepatic failure. All patients in the progression group were preterm birth vs. 37.0 weeks, p vs. 27.30 × 10 9 /L, p = 0.01; CXCL8: 173.8 vs. 34.3 pg/ml, p vs. 209.8 pg/mL, p Conclusion High levels of circulating CXCL8 and CCL2at diagnosis of TAM may predict progressive hepatic failure in DS infants.