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:

Networked: 93 relevant articles (10 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Juvenile Xanthogranuloma
2.	Fatigue
3.	Neoplasms (Cancer)
4.	Langerhans-Cell Histiocytosis (Histiocytosis, Langerhans Cell)
5.	Non-Langerhans-Cell Histiocytosis (Histiocytosis, Non Langerhans Cell)

Experts

1.	Rehák, Z: 5 articles (11/2012 - 01/2011)
2.	Mayer, J: 5 articles (11/2012 - 01/2011)
3.	Zahradová, L: 5 articles (11/2012 - 01/2011)
4.	Szturz, P: 5 articles (11/2012 - 01/2011)
5.	Adam, Z: 5 articles (11/2012 - 01/2011)
6.	Koukalová, R: 5 articles (11/2012 - 01/2011)
7.	Pour, L: 5 articles (11/2012 - 01/2011)
8.	Kurzrock, Razelle: 4 articles (07/2014 - 11/2005)
9.	Hájek, R: 4 articles (11/2012 - 01/2011)
10.	Král, Z: 4 articles (11/2012 - 01/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Erdheim-Chester Disease:

1.	Interferon-alpha (Interferon Alfa)FDA Link 12/01/2001 - "Interferon-alpha can be effective in the treatment of orbital infiltration secondary to Erdheim-Chester disease." 05/01/2009 - "Erdheim-Chester disease with predominant mesenteric localization: lack of efficacy of interferon alpha." 10/01/2006 - "Variability in the efficacy of interferon-alpha in Erdheim-Chester disease by patient and site of involvement: results in eight patients." 09/07/2013 - "[Erdheim-Chester disease: report of a case with retroperitoneal involvement treated with interferon alpha-2a]." 12/13/2011 - "Interferon-alpha in cardiac Erdheim-Chester disease."
2.	Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link 01/01/2014 - "Our experience confirms the efficacy of anakinra for the treatment of Erdheim-Chester disease. " 01/01/2014 - "Within 2 days of starting anakinra, there was prompt resolution of her Erdheim-Chester disease-related symptoms. " 01/01/2014 - "Anakinra, 100 mg subcutaneously daily, was initiated for the treatment of her Erdheim-Chester disease. " 09/01/2013 - "Erdheim-Chester disease with cardiac involvement successfully treated with anakinra." 03/01/2013 - "Favorable radiological outcome of skeletal Erdheim-Chester disease involvement with anakinra."
3.	InterferonsIBA 11/01/2014 - "The field has been particularly active in the last few years, with evidence of the efficacy of interferon-α, the description of a systemic pro-inflammatory cytokine signature, and most recently, reports of the dramatic efficacy of BRAF inhibition in severe, BRAF(V600E) mutation-associated cases of Erdheim-Chester disease. " 06/01/2012 - "Treatment of Erdheim-Chester disease with long-term high-dose interferon-α." 03/10/2011 - "CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients." 12/01/2001 - "Interferon therapy for orbital infiltration secondary to Erdheim-Chester disease." 01/01/2015 - "Rare form of erdheim-chester disease presenting with isolated central skeletal lesions treated with a combination of alfa-interferon and zoledronic Acid."
4.	CladribineFDA LinkGeneric 05/01/2014 - "[PET-CT documented complete remission of Erdheim-Chester disease, lasting more than 4 years from treatment initiation with cladribine]." 05/01/2014 - "Based on case report and publications we consider cladribine as appropriate firs-line drug for Erdheim-Chester disease. " 06/01/2004 - "Treatment of Erdheim-Chester disease with cladribine: a rational approach." 05/01/2014 - "In Pubmed Medline database, we have found 3 publications dedicated to description of treatment response after cladribine in Erdheim-Chester disease and other 7 papers evaluating effect of cladribine on juvenile xanthogranuloma forms, mostly with positive outcome. " 06/01/2012 - "In total, 7 publications describe therapy response of cladribine in some of the juvenile xanthogranuloma forms (Erdheim-Chester disease, disseminated juvenile xanthogranuloma and localized form of plane xanthoma type). "
5.	Interleukin-1 (Interleukin 1)IBA 11/18/2010 - "Rationale and efficacy of interleukin-1 targeting in Erdheim-Chester disease." 03/01/2014 - "We report a new case of successfully interleukin-1 receptor antagonist treatment in Erdheim-Chester disease. " 03/01/2014 - "Successful treatment of Erdheim-Chester disease by interleukin-1 receptor antagonist protein." 03/01/2013 - "We report here a case of interleukin-1 receptor antagonist in the treatment of refractory bones infiltration in Erdheim-Chester disease. " 07/01/2014 - "Historically, the literature regarding the management of Erdheim-Chester disease consisted of case reports and small case series with anecdotal therapeutic responses to agents including, but not limited to, cytotoxic chemotherapy, bone marrow transplantation, cladribine, corticosteroids, IFN-α, the BCR-ABL/KIT inhibitor imatinib mesylate, the IL-1 receptor antagonist anakinra, the TNF-inhibitor infliximab, and recently the BRAF inhibitor vemurafenib. "
6.	Prednisolone (Predate)FDA LinkGeneric 03/01/2008 - "Erdheim-Chester disease was diagnosed in this patient on the basis of diagnostic histopathology, radiologic features involving bones, and ILD and treatment with 40 mg oral prednisolone daily was started. " 01/01/2007 - "[A case of Erdheim-Chester disease effectively treated by cyclophosphamide and prednisolone]." 11/01/2003 - "Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and prednisolone."
7.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2007 - "[A case of Erdheim-Chester disease effectively treated by cyclophosphamide and prednisolone]." 11/01/2003 - "Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and prednisolone."
8.	lenalidomide (CC 5013)FDA Link 01/01/2011 - "Treatment with 2-chlorodeoxyadenosine-based regimen provided partial remission of Erdheim-Chester disease lesions in the brain, while treatment with lenalidomide resulted in complete remission of these lesions. " 01/01/2011 - "[Partial regression of CNS lesions of Erdheim-Chester disease after treatment with 2-chlorodeoxadenosine and their full remission following treatment with lenalidomide]." 11/01/2012 - "[The effect of lenalidomide on rare blood disorders: Langerhans cell histiocytosis, multicentric Castleman disease, POEMS syndrome, Erdheim-Chester disease and angiomatosis]."
9.	Sirolimus (Rapamycin)FDA Link 09/03/2015 - "Sirolimus plus prednisone for Erdheim-Chester disease: an open-label trial."
10.	Prednisone (Sone)FDA LinkGeneric 09/03/2015 - "Sirolimus plus prednisone for Erdheim-Chester disease: an open-label trial."

Therapies and Procedures

1.	Aftercare (After-Treatment) 01/01/2011 - "[Partial regression of CNS lesions of Erdheim-Chester disease after treatment with 2-chlorodeoxadenosine and their full remission following treatment with lenalidomide]."
2.	Radiotherapy 06/01/2007 - "Radiotherapy for Erdheim-Chester disease." 01/01/2015 - "RPF is idiopathic in the majority of cases, but can also be secondary to malignancies, infections, drugs, radiotherapy, and rare histiocytic disorders such as Erdheim-Chester disease. " 09/01/2006 - "A retrospective study of the use of palliative external beam radiotherapy (EBRT) in nine patients with Erdheim-Chester disease was conducted through the Rare Cancer Network. " 09/01/2006 - "Palliative treatment of Erdheim-Chester disease with radiotherapy: a Rare Cancer Network study." 09/01/2002 - "A patient with suprasellar and brain stem involvement in Erdheim Chester disease (ECD) underwent magnetic resonance (MR) imaging and proton MR spectroscopy (1H MRS) of the ventral pons before and 1, 4 and 18 months after external whole-brain (24 Gy) radiotherapy. "
3.	Palliative Care (Palliative Medicine) 08/01/2014 - "Erdheim-Chester disease: from palliative care to targeted treatment." 09/01/2006 - "Palliative treatment of Erdheim-Chester disease with radiotherapy: a Rare Cancer Network study."
4.	Drug Therapy (Chemotherapy) 07/01/2012 - "Bilateral orbital Erdheim-Chester disease treated with 12 weekly administrations of VNCOP-B chemotherapy: a case report and a review of literature." 06/01/2011 - "[Successful treatment of Erdheim-Chester disease by 2-chlorodeoxyadenosine-based chemotherapy. " 10/01/2010 - "Chemotherapy and interferon-alpha treatment of Erdheim-Chester disease." 09/01/2001 - "An administration of systemic corticosteroids, chemotherapy, immunoglobin and traditional Chinese medicine can control Erdheim-Chester disease. " 06/01/2007 - "Erdheim-Chester disease and concomitant tuberculosis successfully treated with chemotherapy and long-term steroids."
5.	Renal Dialysis (Hemodialysis) 10/01/2012 - "Image of Erdheim-Chester disease requiring hemodialysis." 07/01/2012 - "Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis."