A case of erdheim-chester disease with asymptomatic renal involvement

Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis involving bones and multiple organs. Its clinical course can vary, from an asymptomatic state to a fatal disease, with renal involvement being a common cause of death. A 41-year-old man presented with a 10-month history of bilateral...

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Bibliographic Details
Published in:Cancer research and treatment 2012, 44(2), , pp.146-150
Main Authors: Lee, Hyun Jung, Lee, Kyoung Yul, Shin, Dong-Yeop, Lee, Yun Gyoo, Choi, Se Youn, Moon, Kyung Chul, Han, Il-Kyu, Kim, Tae Min
Format: Article
Language:English
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Case Report
의약학
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Summary:Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis involving bones and multiple organs. Its clinical course can vary, from an asymptomatic state to a fatal disease, with renal involvement being a common cause of death. A 41-year-old man presented with a 10-month history of bilateral lower limb pain. Left perirenal soft-tissue infiltration had been found incidentally two years earlier. No progression of the lesion or deterioration of renal function was observed for a period of two years. At admission, plain radiography and magnetic resonance imaging of the patient's lower limbs showed patchy osteosclerosis. Biopsy of the tibia revealed histiocytic infiltration, which was found to be positive for CD68 and negative for CD1a. This report describes an unusual case of Erdheim-Chester disease involving a stationary course of disease with no specific treatment for a long period of time.
ISSN:1598-2998
2005-9256
DOI:10.4143/crt.2012.44.2.146