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Journal of the Korean Society of Neonatology 2003;10(1):83-87.
Published online May 1, 2003.
Down Syndrome with Transient Myeloproliferative Disorder, Hepatic Fibrosis, and Hemochromatosis.
Jie Yeon Lee, Hwi Kyu Im, Hwang Min Kim, Baek Keun Lim, Young Uh, Chan Il Park
1Department of Pediatrics, Wonju Medical College, Yonsei University, Wonju, Korea. limbk@kornet.net
2Department of Laboratory Medicine, Wonju Medical College, Yonsei University, Wonju, Korea.
3Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
일과성 골수증식장애, 간섬유증 및 혈색소증이 동반된 다운증후군 신생아 1례
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Transient myeloproliferative disorder (TMD), which may mimic acute leukemia, occurs in neonates with Down syndrome along with hepatic fibrosis. TMD is recognized shortly after birth or in the neonatal period and is characterized by leukocytosis and thrombocytopenia, which resolve spontaneously in four to six weeks. And hepatic fibrosis is characterized by diffuse intralobular sinusoidal fibrosis, extramedullary hematopoiesis and hemochromatosis. A newborn male infant with Down syndrome, atrial septal defect and ventricular septal defect is reported. He showed abnormal myelopoiesis accompanying characteristic hepatic sinusoidal fibrosis. Knowing the cellular mechanism of hepatic fibrosis and its modulation by growth factors, a pathogenetic link between transient myeloproliferative disorder and the development of liver fibrosis in Down syndrome neonates, association of this triad no longer appears to be accidental.
Key Words: Down syndrome; Transient myeloproliferative disorder; Hepatic fibrosis; Hemochromatosis


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