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Lege Artis Medicinae

MAY 16, 2007

[ERDHEIM-CHESTER DISEASE: A RARE FORM OF HISTIOCYTOSIS]

NÉMETH István, BOGÁTS Gábor, KAISER László, TISZLAVICZ László

[INTRODUCTION - Erdheim-Chester disease is rare non-Langerhans cell histiocytosis with multisystem involvement. The histological hallmark of the disease is CD1a negative histiocyte proliferation. CASE REPORT - Histiocytosis presented in a 57- year-old Caucasian man with exophtalmus, which was after two years followed by progressive pericardial infiltration and effusion leading to cardiac compression and consequent functional disturbance. It was the histological analysis of a surgical sample removed during pericardial fenestration that revealed Erdheim- Chester disease. The patient died from multisystem involvement one year after the initial diagnosis. Postmortem examination showed long bone, orbital cavity and cranial cavity involvement as well as extensive retroperitoneal and mediastinal xanthogranulomatous infiltration. CONCLUSIONS - The clinical diagnosis of Erdheim-Chester disease is difficult because the symptoms are insidious and non-specific. The histological report is often only descriptive. The prognosis of the disease is poor due to the lack of targeted therapy and to the advanced stage at diagnosis.]