Hematopoietic origin of Langerhans cell histiocytosis and Erdheim-Chester disease in adults

Author:

Milne Paul1,Bigley Venetia1,Bacon Chris M.23,Néel Antoine4,McGovern Naomi1,Bomken Simon2,Haniffa Muzlifah1,Diamond Eli L.5,Durham Benjamin H.5,Visser Johannes6,Hunt David7,Gunawardena Harsha8,Macheta Mac9,McClain Kenneth L.10,Allen Carl10,Abdel-Wahab Omar5,Collin Matthew1

Affiliation:

1. Institute of Cellular Medicine and

2. Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne, United Kingdom;

3. Department of Cellular Pathology, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, United Kingdom;

4. Internal Medicine Department, Hôtel-Dieu University Hopital, Nantes, France;

5. Memorial Sloan Kettering Cancer Center, New York, NY;

6. East Midlands Children’s and Young Persons’ Integrated Cancer Service, Leicester Children’s Hospital, Leicester, United Kingdom;

7. Medical Research Council Institute of Genetics and Molecular Medicine, University of Edinburgh, Edinburgh, United Kingdom;

8. Rheumatology Department, North Bristol National Health Service Trust, Bristol, United Kingdom;

9. Blackpool Teaching Hospitals National Health Service Foundation Trust, Blackpool, United Kingdom; and

10. Texas Children’s Cancer Center, Baylor College of Medicine, Houston, TX

Abstract

Key Points Bone marrow progenitors, monocytes, and myeloid DCs contain BRAFV600E alleles in adults with LCH and ECD. Mutant allele distribution is not disease specific, but precursors have distinct LCH-like and macrophage differentiation capacities.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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