Life-threatening complications of transient abnormal myelopoiesis in neonates with Down syndrome.
Neonates with Down syndrome can present with a haematological disorder called transient abnormal myelopoiesis (TAM). While TAM is usually a self-limiting disease, patients with severe complications such as hydrops fetalis, cardiorespiratory failure and liver fibrosis have been described. Here, we present five consecutive neonates with trisomy 21 and TAM, four of whom were critically ill and were therefore treated with cytosine-arabinoside. All five patients survived.


CONCLUSIONS
severely affected neonates with Down syndrome and transient abnormal myelopoiesis might benefit from early cytostatic treatment with cytosine-arabinoside.
Dormann
Sabine
S
Department of Paediatrics and Adolescent Medicine, University of Freiburg, Mathildenstrasse 1, 79106 Freiburg, Germany. dormann@kikli.ukl.uni-freiburg.de
Krüger
Marcus
M
Hentschel
Roland
R
Rasenack
Regina
R
Strahm
Brigitte
B
Kontny
Udo
U
Niemeyer
Charlotte
C
D001772:Blood Cell Count; D001773:Blood Cells / Q000187:drug effects / Q000378:metabolism; D003561:Cytarabine / Q000627:therapeutic use; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006402:Hematologic Diseases / Q000209:etiology*; D006801:Humans; D015160:Hydrops Fetalis / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007231:Infant, Newborn; D008103:Liver Cirrhosis / Q000209:etiology; D008297:Male; D038042:Myelopoiesis; D012131:Respiratory Insufficiency / Q000209:etiology; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D003561:Cytarabine