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

Gabalec, Filip; Šimkovič, Martin; Zavřelová, Alžběta; Kašparová, Petra; Morávková, Paula; Kopáčová, Marcela; Masopust, Václav; Žák, Pavel; Čáp, Jan; Radocha, Jakub

doi:10.14712/18059694.2018.11

Acta Medica > Archive > 2017, Vol. 60 > Issue 4 > Treatment of Multifocal Multisystem BRAF…

Acta Med. 2017, 60: 152-156

https://doi.org/10.14712/18059694.2018.11

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

Filip Gabalec^a

, Martin Šimkovič^a

, Alžběta Zavřelová^a, Petra Kašparová^b

, Paula Morávková^c

, Marcela Kopáčová^c, Václav Masopust^d

, Pavel Žák^a, Jan Čáp^a, Jakub Radocha^a

^a4th Department of Internal Medicine – Haematology, University Hospital Hradec Králové, Czech Republic and Charles University, Faculty of Medicine in Hradec Králové, Czech Republic
^bFingerland’s Department of Pathology, University Hospital Hradec Králové, Czech Republic and Charles University, Faculty of Medicine in Hradec Králové, Czech Republic
^c2nd Department of Internal Medicine – Gastroenterology, University Hospital Hradec Králové, Czech Republic and Charles University, Faculty of Medicine in Hradec Králové, Czech Republic
^dNeurosurgery Clinic, Central Military Hospital Prague, Prague, Czech Republic

Received October 24, 2017
Accepted December 20, 2017

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.