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Revista Argentina de Hematología

Abstract

Volumen:    21    # Number : 1

Publication Date :    Enero - Abril    Year:    2017

   ARTÍCULO ORIGINAL

Erdheim Chester disease: report of two cases

Authors: López J, Paganini M, Juncosa V, Ballestracci M, Espejo Fusari G, Aberastain A, Salomon J, Alume J, Capitani R, Viani de Ditter V, Espejo Cozzi A.

Abstract: Erdheim-Chester disease (ECD) is a rare, systemic, non-Langerhans cell histiocytosis. Clinical manifestations vary depending on the organ systems involved. Radiologically, the affectation of long bones by symmetrical metadisphysary sclerosis is characteristic. The infiltration of central nervous system is an independent poor prognosis factor. There are few reports in the medical literature. We discuss two cases because of the infrequency of the disease.

Key words: Erdheim-Chester, non-Langerhans histiocytosis, osteosclerosis.

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