review
Recent advances in the understanding of Langerhans cell histiocytosis
Gayane Badalian-Very,
Jo-Anne Vergilio,
Barbara A. Degar,
Carlos Rodriguez-Galindo,
Barrett J. Rollins,
Gayane Badalian-Very
Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA
Department of Medicine, Brigham & Women’s Hospital
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorJo-Anne Vergilio
Department of Pathology, Children’s Hospital Boston, Boston, MA
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorBarbara A. Degar
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA
Departments of Medicine, Children’s Hospital Boston
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorCarlos Rodriguez-Galindo
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA
Departments of Medicine, Children’s Hospital Boston
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorBarrett J. Rollins
Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA
Department of Medicine, Brigham & Women’s Hospital
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorGayane Badalian-Very,
Jo-Anne Vergilio,
Barbara A. Degar,
Carlos Rodriguez-Galindo,
Barrett J. Rollins,
Gayane Badalian-Very
Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA
Department of Medicine, Brigham & Women’s Hospital
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorJo-Anne Vergilio
Department of Pathology, Children’s Hospital Boston, Boston, MA
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorBarbara A. Degar
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA
Departments of Medicine, Children’s Hospital Boston
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorCarlos Rodriguez-Galindo
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA
Departments of Medicine, Children’s Hospital Boston
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorBarrett J. Rollins
Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA
Department of Medicine, Brigham & Women’s Hospital
Departments of Medicine, Pathology, and Pediatrics, Harvard Medical School, Boston, MA, USA
Search for more papers by this authorBarrett J. Rollins, MD, PhD, Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02215, USA. E-mail: [email protected]
Summary
Langerhans cell histiocytosis (LCH) is a proliferative disease of cells that share phenotypic characteristics with the primary antigen presenting cells of the epidermis. Its clinical manifestations are highly variable, extending from very benign forms to a disseminated, aggressive disease that causes significant mortality. Although many of the fundamental pathogenetic features of LCH have been enigmatic, recent advances have led to a much clearer understanding of the disease. In particular, careful molecular analyses of mouse models and human LCH samples suggest that LCH’s cell of origin may not be the epidermal LC itself but a myeloid-derived precursor. Advanced genomic technologies have revealed the presence of activating, somatic BRAF mutations in the majority of patient specimens. Together, these observations have produced a new picture of LCH as a myeloid neoplasm. These advances are likely to have profound implications for the use of targeted therapeutics in LCH.
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