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International Journal of Hematology

, Volume 99, Issue 2, pp 154–161 | Cite as

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

  • Myoung Ja ParkEmail author
  • Manabu Sotomatsu
  • Kentaro Ohki
  • Kokoro Arai
  • Kenichi Maruyama
  • Tomio Kobayashi
  • Akira Nishi
  • Kiyoko Sameshima
  • Takeshi Takagi
  • Yasuhide Hayashi
Original Article

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.

Keywords

Down syndrome AML Liver disease Direct bilirubin Cytarabine therapy 

Notes

Acknowledgments

This work was supported by a grant for Cancer Research, and a grant for Research on Children and Families from the Ministry of Health, Labor, and Welfare of Japan, and a Grant-in-Aid for Scientific Research (B) and (C) and Exploratory Research from the Ministry of Education, Culture, Sports, Science, and Technology of Japan, and a Research grant for Bureau of Gunma Prefectural Hospitals.

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Copyright information

© The Japanese Society of Hematology 2013

Authors and Affiliations

  • Myoung Ja Park
    • 1
    Email author
  • Manabu Sotomatsu
    • 1
  • Kentaro Ohki
    • 1
  • Kokoro Arai
    • 1
  • Kenichi Maruyama
    • 2
  • Tomio Kobayashi
    • 3
  • Akira Nishi
    • 4
  • Kiyoko Sameshima
    • 5
  • Takeshi Takagi
    • 6
  • Yasuhide Hayashi
    • 1
  1. 1.Department of Hematology/OncologyGunma Children’s Medical CenterShibukawaJapan
  2. 2.Department of NeonatologyGunma Children’s Medical CenterGunmaJapan
  3. 3.Department of CardiologyGunma Children’s Medical CenterGunmaJapan
  4. 4.Department of Pediatric SurgeryGunma Children’s Medical CenterGunmaJapan
  5. 5.Department of GeneticsGunma Children’s Medical CenterGunmaJapan
  6. 6.Department of ObstetricsGunma Children’s Medical CenterGunmaJapan

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