Кладрибин в лечении многоочагового мультисистемного гистиоцитоза из клеток Лангерганса у пациента с неблагоприятным прогнозом. Клиническое наблюдение

Abstract

Langerhans cell histiocytosis is a hematologic clonal disease. The treatment of localized histiocytosis includes local methods such as radiotherapy or surgery. In cases of disseminated lesions with multiple system damage, chemotherapy is administered, which provides recovery in some patients. The present paper is a case report of multifocal multisystem Langerhans cell histiocytosis with bone, liver, spleen, abdominal lymph node and pituitary lesions in a 40-year-old patient. The diagnosis was verified by histology and immunohistochemistry of bone biopsy specimens. Polymerase chain reaction revealed no V600E mutation in the BRAF gene in tumor tissue. Persistent antitumor response was achieved after 6 chemotherapy cycles with cladribine monoregimen. This drug was well tolerated by the patient. By the time of this publication, the patient had preserved the persistent response for 38 months.