Erdheim Chester Disease Mimicking Lymphoma: A Case Report

Background

Erdheim Chester disease (ECD) is a rare disorder, characterized by organ infiltration of non-Langerhans cell foamy, CD68⁺, CD1a^- histiocytes. Since first described in 1930 by William Chester, > 900 cases have been reported. The disease usually affects patients in the 5^th – 7^th decade of life with a predilection for males [1] [2] [3].

Publication History

Article published online:
14 October 2021

• References

• 1 Campochiaro C, Tomelleri A, Cavalli G. et al. Erdheim-Chester disease. Eur J Intern Med 2015; 26: 223-229 DOI: 10.1016/j.ejim.2015.03.004.
• 2 Cives M, Simone V, Rizzo FM. et al. Erdheim-Chester disease: A systematic review. Crit Rev Oncol Hematol 2015; 95: 1-11 DOI: 10.1016/j.critrevonc.2015.02.004.
• 3 Goyal G, Heaney ML, Collin M. et al. Erdheim-Chester disease: Consensus recommendations for evaluation, diagnosis, and treatment in the molecular era. Blood 2020; 135: 1929-1945 DOI: 10.1182/blood.2019003507.
• 4 Salama HA, Jazieh AR, Alhejazi AY. et al. Highlights of the Management of Adult Histiocytic Disorders: Langerhans Cell Histiocytosis, Erdheim-Chester Disease, Rosai-Dorfman Disease, and Hemophagocytic Lymphohistiocytosis. Clin Lymphoma, Myeloma Leuk 2021; 21: e66-e75 DOI: 10.1016/j.clml.2020.08.007.
• 5 Diamond EL, Dagna L, Hyman DM. et al. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood 2014; 124: 483-492 DOI: 10.1182/blood-2014-03-561381.
• 6 Arnaud L, Hervier B, Ne A. et al. CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients. 2011; 117: 2778-2782 DOI: 10.1182/blood-2010-06-294108.
• 7 Berti A, Ferrarini M, Ferrero E. et al. Review Cardiovascular manifestations of Erdheim-Chester disease. 2009: 8-10
• 8 Sheu SY. Erdheim-Chester disease: case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literature. J Clin Pathol 2004; 57 DOI: 10.1136/jcp.2004.018481.
• 9 Sung YE, Lee YS, Lee J. et al. Erdheim-Chester disease involving lymph nodes and liver clinically Mimicking Lymphoma: A case report. J Pathol Transl Med 2018; 52: 183-190 DOI: 10.4132/jptm.2017.10.16.
• 10 Lim J, Kim KH, Suh KJ. et al. A Unique Case of Erdheim-Chester Disease with Axial Skeleton, Lymph Node, and Bone Marrow Involvement. Cancer Res Treat 2016; 48 DOI: 10.4143/crt.2014.160.
• 11 Sakr HI, Buckley K, Baiocchi R. et al. Erdheim Chester disease in a patient with Burkitt lymphoma: A case report and review of literature. Diagn Pathol 2018; 13: 1-6 DOI: 10.1186/s13000-018-0772-2.
• 12 Maietta A, Razmpoosh M. Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass. Case Rep Pathol 2020; 2020: 1-6 DOI: 10.1155/2020/3081824.