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Erdheim-chester disease associated with marginal zone lymphoma and monoclonal proteinemia

Peter G Pavlidakey1, Alok Mohanty, Lisa J Kohler

  • 1Department of Pathology, University Hospitals Case Medical Center and Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106-5056, USA.

Case Reports in Hematology
|September 1, 2012
PubMed

Insights

Erdheim-Chester disease (ECD), a rare histiocytosis, can present with cardiac issues. This case highlights a fatal ECD presentation with lymphoma and monoclonal proteinemia, a previously unreported association.

Area of Science:

  • Histiocytosis research
  • Cardiovascular pathology
  • Oncohematology

Background:

  • Erdheim-Chester disease (ECD) is an exceedingly rare non-Langerhans cell histiocytosis.
  • Cardiac involvement in ECD is uncommon and typically presents with pericardial effusion or thickening.

Observation:

  • A young male patient presented with Erdheim-Chester disease.
  • The patient exhibited extensive cardiac infiltration by ECD.
  • Co-existing conditions included marginal zone lymphoma and monoclonal proteinemia.

Findings:

  • This case represents the first documented association between Erdheim-Chester disease and monoclonal gammopathy.
  • The co-occurrence of ECD with lymphoma has not been previously reported.
  • Extensive cardiac involvement significantly contributed to the fatal outcome.

Implications:

  • This case expands the known clinical spectrum of Erdheim-Chester disease.
  • It suggests a potential link between ECD and lymphoproliferative disorders.
  • Further research is warranted to explore the pathogenesis and potential shared mechanisms between ECD, monoclonal gammopathies, and lymphomas.

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