Treatment of Multifocal Multisystem BRAF Positive Langerhans Cell Histiocytosis with Cladribine, Surgery and Allogenic Stem Cell Transplantation
Language English Country Czech Republic Media print
Document type Case Reports, Journal Article
Grant support
PROGRES Q40/08
Univerzita Karlova v Praze
00179906
Ministerstvo Zdravotnictví Ceské Republiky
PubMed
29716681
DOI
10.14712/18059694.2018.11
PII: am_2017060040152
Knihovny.cz E-resources
- Keywords
- cladribine, histiocytosis, hypopituitarism, stem cell, transplantation,
- MeSH
- Biopsy methods MeSH
- Adult MeSH
- Histiocytosis, Langerhans-Cell * diagnosis genetics physiopathology therapy MeSH
- Pituitary Gland * diagnostic imaging pathology MeSH
- Immunosuppressive Agents administration & dosage MeSH
- Cladribine administration & dosage MeSH
- Humans MeSH
- Magnetic Resonance Imaging methods MeSH
- Proto-Oncogene Proteins B-raf genetics MeSH
- Severity of Illness Index MeSH
- Stem Cell Transplantation methods MeSH
- Treatment Outcome MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Names of Substances
- BRAF protein, human MeSH Browser
- Immunosuppressive Agents MeSH
- Cladribine MeSH
- Proto-Oncogene Proteins B-raf MeSH
Langerhans cell histiocytosis (LCH) is a very rare disease in adults and as well a very rare cause of sellar expansion. The clinical presentation can be heterogeneous, from a single bone lesion to potentially fatal, widespread disease. We describe the difficulties with the diagnosis and treatment of LCH as well as successful treatment with cladribine chemotherapy and allogeneic stem cell transplantation.
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