Treatment of Multifocal Multisystem BRAF Positive Langerhans Cell Histiocytosis with Cladribine, Surgery and Allogenic Stem Cell Transplantation

Acta Medica (Hradec Kralove). 2017;60(4):152-156. doi: 10.14712/18059694.2018.11.

Abstract

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.

Keywords: cladribine; histiocytosis; hypopituitarism; stem cell; transplantation.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy / methods
  • Cladribine / administration & dosage*
  • Female
  • Histiocytosis, Langerhans-Cell* / diagnosis
  • Histiocytosis, Langerhans-Cell* / genetics
  • Histiocytosis, Langerhans-Cell* / physiopathology
  • Histiocytosis, Langerhans-Cell* / therapy
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Magnetic Resonance Imaging / methods
  • Pituitary Gland* / diagnostic imaging
  • Pituitary Gland* / pathology
  • Proto-Oncogene Proteins B-raf / genetics*
  • Severity of Illness Index
  • Stem Cell Transplantation / methods*
  • Treatment Outcome

Substances

  • Immunosuppressive Agents
  • Cladribine
  • BRAF protein, human
  • Proto-Oncogene Proteins B-raf