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Erdheim-Chester Disease

A rare form of non-Langerhans-cell histiocytosis (HISTIOCYTOSIS, NON-LANGERHANS-CELL) with onset in middle age. The systemic disease is characterized by infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.
Also Known As:
Erdheim Chester Disease; Lipid Granulomatosis; Granulomatosis, Lipid
Networked: 272 relevant articles (32 outcomes, 9 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Langerhans-Cell Histiocytosis (Histiocytosis, Langerhans Cell)
2. Neoplasms (Cancer)
3. Juvenile Xanthogranuloma
4. Melanoma (Melanoma, Malignant)
5. Renal Insufficiency (Renal Failure)

Experts

1. Haroche, Julien: 25 articles (01/2022 - 10/2006)
2. Amoura, Zahir: 21 articles (01/2022 - 10/2006)
3. Emile, Jean-François: 15 articles (11/2020 - 02/2013)
4. Cohen-Aubart, Fleur: 11 articles (01/2022 - 02/2013)
5. Diamond, Eli L: 11 articles (01/2021 - 08/2015)
6. Dagna, Lorenzo: 10 articles (01/2022 - 10/2012)
7. Charlotte, Frédéric: 9 articles (11/2019 - 03/2011)
8. Cavalli, Giulio: 8 articles (01/2022 - 01/2014)
9. Maksud, Philippe: 8 articles (10/2021 - 02/2013)
10. Go, Ronald S: 7 articles (01/2022 - 09/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Erdheim-Chester Disease:
1. VemurafenibIBA
2. Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link
3. Interferon-alpha (Interferon Alfa)IBA
4. Interleukin-1 (Interleukin 1)IBA
5. InterferonsIBA
6. CladribineFDA LinkGeneric
7. CytokinesIBA
8. Interleukin-1 Receptors (Interleukin 1 Receptor)IBA
9. dabrafenibIBA
10. Mitogen-Activated Protein Kinase Kinases (MEKs)IBA

Therapies and Procedures

1. Therapeutics
2. Renal Dialysis (Hemodialysis)
3. Aftercare (After-Treatment)
4. Kidney Transplantation
5. Radiotherapy