The clinical spectrum of Erdheim-Chester disease: an observational cohort study

Author:

Estrada-Veras Juvianee I.1ORCID,O’Brien Kevin J.2,Boyd Louisa C.1ORCID,Dave Rahul H.3ORCID,Durham Benjamin H.4ORCID,Xi Liqiang5,Malayeri Ashkan A.6,Chen Marcus Y.7ORCID,Gardner Pamela J.8,Alvarado Enriquez Jhonell R.1,Shah Nikeith1,Abdel-Wahab Omar910ORCID,Gochuico Bernadette R.1,Raffeld Mark5,Jaffe Elaine S.5ORCID,Gahl William A.12

Affiliation:

1. Section on Human Biochemical Genetics, Medical Genetics Branch, and

2. Office of the Clinical Director, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD;

3. Viral Immunology and Intravital Imaging Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD;

4. Department of Pathology, Mortimer B. Zuckerman Research Center, Memorial Sloan Kettering Cancer Center, New York, NY;

5. Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD;

6. Laboratory of Diagnostic Radiology Research, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD;

7. Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD;

8. Dental Consult Service, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD; and

9. Human Oncology and Pathogenesis Program and

10. Leukemia Service, Department of Medicine, Mortimer B. Zuckerman Research Center, Memorial Sloan Kettering Cancer Center, New York, NY

Abstract

Key Points ECD varies in terms of age of onset, clinical presentation, manifestations, organ involvement, disease severity, and survival. ECD is a neoplasm and should be adopted by the field of hematology-oncology following the World Health Organization reclassification.

Publisher

American Society of Hematology

Subject

Hematology

Reference34 articles.

1. Uber Lipoidgranulomatose;Chester;Virchows Arch Pathol Anat.,1930

2. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages;Emile;Blood,2016

3. Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases;Veyssier-Belot;Medicine (Baltimore),1996

4. Erdheim-Chester disease;Haroche;Curr Rheumatol Rep,2014

5. Erdheim-Chester disease;Campochiaro;Eur J Intern Med,2015

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