Reduced-intensity conditioned allogeneic haematopoietic transplantation in an adult with Langerhans' cell histiocytosis and thrombocytopenia with absent radii.

摘要

Langerhans cell histiocytosis (LCH) is a rare disorder in adults, presenting as single organ or multisystem disease, with pulmonary disease occurring more frequently in isolation.1 Failure of chemotherapy is reported in 20% of cases with multisystem LCH with overall survival of only 20%.2-3 Reports of myeloablative allogeneic stem cell transplantation in isolated cases of refractory LCH in children are described.4-5 A high transplant-related mortality, particularly in the presence of pre-existing end-organ damage, limits its use.