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Revista chilena de pediatría

versión impresa ISSN 0370-4106

Resumen

TORDECILLA C, Juan; RODRIGUEZ Z, Natalie; ALVAREZ A, Patricia  y  VELOZO P, Luis. Transient myeloproliferation and hepatic fibrosis in Down’s Syndrome. Rev. chil. pediatr. [online]. 2003, vol.74, n.1, pp.64-69. ISSN 0370-4106.  http://dx.doi.org/10.4067/S0370-41062003000100009.

Transient myeloproliferation occurs in up to 10% of newborns with Down’s syndrome, and in most cases resolves spontaneously during the first 3 months of life. It is characterised by a hyperleucocytosis, blasts in the peripheral blood and bone marrow, hepatosplenomegaly, heart disease and 20% will progress to severe hepatic fibrosis with a consequent high mortality. We present a case of a newborn with Down's syndrome with hepatosplenomegaly, anaemia, hrombocitopenia, hyperleucocitosis, peripheral blasts, a patent ductus arteriosus and progressive liver disease. An alternative diagnosis of TORCH or metabolic disease were excluded. Bone marrow aspiration showed 43% FAB M/ type blasts. Liver biopsy showed deranged architecture, pseudoacinar transformation, diffuse fibrosis, hepatocellular and canalicular cholestasis, haemosiderosis and haematopoiesis. The patient died of multiple organ failure 66 days after birth. We review the pathophysiological and genetic aspects of transient myeloproliferation and suggest the use of chemotherapy in cases of severe liver disease

Palabras clave : Down’s syndrome; transient myeloproliferation; hepatic fibrosis.

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