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Journal of the Korean Society of Neonatology 2003;10(1):78-82.
Published online May 1, 2003.
A Case of Transient Myeloproliferative Disorder with Down Syndrome, Presented Hepatosplenomegaly on Prenatal Sonography.
Keun Hye Lee, Beom Su Park, Eun Hwan Jeong, Bo Ra Son, Mi Kyeong Kim, Seung Woon Lim, Hyeon Jin Park
1Department of Pediatrics, College of Medicine, Chung Buk National University. hjpark@chungbuk.ac.kr
2Department of Obstetrics, College of Medicine, Chung Buk National University.
3Department of Clinical Pathology, College of Medicine, Chung Buk National University.
4Department of Internal Medicine, College of Medicine, Chung Buk National University.
5Department of Anesthesiology, College of Medicine, Chung Buk National University.
산전 초음파상 간비종대를 보였던 일과성 골수증식성 장애를 동반한 다운 증후군 환아 1례
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Abstract
Down syndrome (DS) is associated with a higher incidence of leukemia than general population; the subtype is acute megakaryoblastic leukemia (AMKL) in 50% of cases. DS is also strongly associated with transient myeloproliferative disorder (TMD), which is usually diagnosed during newborns and infants. Due to its difficulty in distinguishing TMD from acute leukemia (AL), the diagnosis of TMD should be made with extreme caution. Unlike AL, most cases of TMD resolve spontaneously within 3 months; blast cells disappear within 8 weeks in 80% and within 10 weeks in 90% of the surviving patients. Some infants with TMD, however, may have a severe complication leading into life-threatening clinical course with hepatosplenomegaly, lymphadenopathy, liver impairment, respiratory distress, anemia, infection and hemorrhage. Rarely, AL can develop after remission of TMD. We report a case of TMD with DS in newly born infant who presented hepatosplenomegaly on prenatal sonography and improved with exchange transfusion, steroid.
Key Words: Transient myeloproliferative disorder, Down syndrome, Hepatosplenomegaly, Prenatal sonography


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