A Case of Langerhans Cell Histiocytosis With Risk Organ Involvement in a Young Adult

Abstract

Multisystem Langerhans cell histiocytosis (MS-LCH) is a rare disease for which the standard of care has not been clearly established. We are the first to address frontline and salvage options for the management of an adolescent young adult (AYA) male with MS-LCH with "risk organ" involvement, a population for which there are no therapeutic guidelines. Our 33-year-old male patient presented with MS-LCH with generalized lymphadenopathy, hepatic and splenic involvement. He was initially treated with vinblastine and prednisolone with progression in disease. Our exploration of the literature revealed that salvage options in adults include single-agent chemotherapy, hematopoietic stem cell transplant, imatinib, and vemurafenib and are limited to small case series with questionable efficacy. We opted to use a pediatric regimen with the combination of cladribine and cytarabine and demonstrate that this approach can in fact be effective in an AYA patient.