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

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

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

Číslo: 4/2017
Periodikum: Acta Medica
DOI: 10.14712/18059694.2018.11

Klíčová slova: histiocytosis, transplantation, stem cell, cladribine, hypopituitarism, histiocytóza, transplantace, kmenové buňky, kladribín, hypopituitarismus

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Anotace: 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.