ArticleJournal of pathology and translational medicine2018
Erdheim-Chester Disease Involving Lymph Nodes and Liver Clinically Mimicking Lymphoma: A Case Report.
Article in Journal of pathology and translational medicine, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
What it found
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Who cites it
7 citing papers in PubMed, 13 citations in OpenAlex.
- Case Report: Erdheim-Chester disease with liver involvement successfully treated with trametinib.Frontiers in oncology · 2026Article
- Pathologic characteristics of histiocytic and dendritic cell neoplasms.Blood research · 2024Review
- Dramatic Response After Switching MEK Inhibitors in a Patient With Refractory Mixed Histiocytosis.Journal of hematology · 2022Article
- Erdheim-Chester Disease with Isolated CNS Involvement: A Systematic Review of the Literature.Neurology international · 2022Review
- Erdheim-Chester disease with long-standing diabetes insipidus and generalized edema.Clinical case reports · 2021Article
- Primary Necrobiotic Xanthogranulomatous Sialadenitis with Submandibular Gland Localization without Skin Involvement.Journal of pathology and translational medicine · 2019Article
- [Erdheim-Chester disease : An important differential diagnosis and its main symptoms].Zeitschrift fur Rheumatologie · 2019Review
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis and multisystem disease. First described in 1930, there are no more than 750 cases reported. The etiology remains unknown, but a majority of cases of ECD and Langerhans cell histiocytosis were found to have clonal mutations involving genes of the mitogen-activated protein kinase pathway. We recently encountered a 53-year-old male patient with extensive ECD involving the systemic lymph nodes, pleura, liver, and long bones clinically mimicking malignant lymphoma. Biopsies were performed at multiple sites, including a pleural mass, an external iliac lymph node, bone marrow, and the liver. Based on histopathological and immunohistochemical findings of positivity for CD68 and negativity for CD1a and S-100, the patient was diagnosed with ECD. Interferon-α was administered as the first-line treatment, but the patient rapidly progressed to hepatic failure after 2 months of treatment. We report this rare case of ECD clinically mimicking malignant lymphoma and diagnosed by careful pathological review.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.