Hydrops fetalis in a neonate with down syndrome, transient myeloproliferative disorder and hepatic fibrosis.
Transient myeloproliferative disorder is a self limiting disorder characterized by leukocytosis with the presence of megakaryoblasts in the peripheral blood and bone marrow, anemia, thrombocytopenia, and organomegaly. It occurs in approximately 10% of newborn infants with Down syndrome. Hepatic fibrosis is seen in the severe form of transient myeloproliferative disorder with Down syndrome that is characterized by diffuse intralobular sinusoidal fibrosis and extramedullary hematopoesis. We describe a patient with hydrops fetalis, Down syndrome, and transient myeloproliferative disorder. We suggest that patients with the severe form of transient myeloproliferative disorder should be examined for hepatic fibrosis.
Anuk
Deniz
D
Department of Pediatrics, Baskent University Faculty of Medicine, Ankara, Turkey. denizanuk@yahoo.com
Tarcan
Aylin
A
Alioglu
Bulent
B
Avci
Zekai
Z
Haberal
Nihan
N
Ozyurek
Emel
E
Ozbek
Namik
N
D004314:Down Syndrome / Q000150:complications* / Q000503:physiopathology; D017809:Fatal Outcome; D006801:Humans; D015160:Hydrops Fetalis / Q000209:etiology* / Q000503:physiopathology; D007231:Infant, Newborn; D007234:Infant, Premature; D008103:Liver Cirrhosis / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000503:physiopathology