HYDROPS FETALIS IN A NEONATE WITH DOWN SYNDROME, TRANSIENT MYELOPROLIFERATIVE DISORDER AND HEPATIC FIBROSIS |
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Authors: | Deniz Anuk Aylin Tarcan Bulent Alioglu Zekai Avci Nihan Haberal Emel Ozyurek Namik Ozbek |
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Affiliation: | a Department of Pediatrics, Baskent University Faculty of Medicine, Ankara, Turkeyb Department of Neonatology, Baskent University Faculty of Medicine, Ankara, Turkeyc Department of Pediatric Hematology, Baskent University Faculty of Medicine, Ankara, Turkeyd Department of Pathology, Baskent University Faculty of Medicine, Ankara, Turkey |
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Abstract: | 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. |
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Keywords: | transient myeloproliferative disorder Down syndrome hepatic fibrosis newborn |
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