A Case of Transient Myeloproliferative Disorder with Down Syndrome, Presented Hepatosplenomegaly on Prenatal Sonography.
- Author:
Keun Hye LEE
1
;
Beom Su PARK
;
Eun Hwan JEONG
;
Bo Ra SON
;
Mi Kyeong KIM
;
Seung Woon LIM
;
Hyeon Jin PARK
Author Information
1. Department of Pediatrics, College of Medicine, Chung Buk National University. hjpark@chungbuk.ac.kr
- Publication Type:Case Report
- Keywords:
Transient myeloproliferative disorder;
Down syndrome;
Hepatosplenomegaly;
Prenatal sonography
- MeSH:
Anemia;
Diagnosis;
Down Syndrome*;
Hemorrhage;
Humans;
Incidence;
Infant;
Infant, Newborn;
Leukemia;
Leukemia, Megakaryoblastic, Acute;
Liver;
Lymphatic Diseases;
Myeloproliferative Disorders*
- From:Journal of the Korean Society of Neonatology
2003;10(1):78-82
- CountryRepublic of Korea
- Language:Korean
-
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.
