Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. We report a fatal case of ECD with extensive cardiac involvement associated with a marginal zone lymphoma and monoclonal proteinemia in a young man. This is the first reported association of ECD with a monoclonal gammopathy or a lymphoma.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3420507PMC
http://dx.doi.org/10.1155/2011/941637DOI Listing

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