Liver disease is frequently observed in Down syndrome patients with transient abnormal myelopoiesis

International Journal of Hematology
Myoung Ja ParkYasuhide Hayashi

Abstract

Transient abnormal myelopoiesis (TAM) in neonates with Down syndrome (DS) is characterized by the transient appearance of blast cells, which resolves spontaneously. Approximately 20 % of patients with TAM die at an early age due to organ failure, including liver disease. We studied 25 DS-TAM patients retrospectively to clarify the correlation between clinical and laboratory characteristics and liver diseases. Early death (<6 months of age) occurred in four of the 25 patients (16.0 %), and two of those four patients died due to liver failure. Although physiologic jaundice improved gradually after a week, all DS patients had elevated D-Bil levels during the clinical course of TAM, except one who suffered early death. The median peak day of the WBC count, total bilirubin (T-Bil) and D-Bil levels was: day 1 (range day 0-57), day 8 (range day 1-55), and day 17 (range 1-53), respectively. Our results reveal that all patients with DS-TAM may develop liver disease irrespective of the absence or presence of symptoms and risk factors for early death. In patients of DS-TAM, careful observation of the level of D-Bil is needed by at least 1 month of age for the detection of liver disease risk.

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Citations

Aug 12, 2016·Current Hematologic Malignancy Reports·Neha BhatnagarIrene Roberts
Aug 23, 2016·The Tohoku Journal of Experimental Medicine·Hajime MaedaMitsuaki Hosoya
Jun 20, 2018·British Journal of Haematology·Oliver TunstallUNKNOWN British Society for Haematology
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Dec 30, 2018·Pediatrics International : Official Journal of the Japan Pediatric Society·Kenichiro Watanabe
Apr 13, 2017·Pediatrics International : Official Journal of the Japan Pediatric Society·Toshihiko WatanabeYutaka Kanamori
Jan 5, 2021·International Journal of Hematology·Genki YamatoYasuhide Hayashi

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