Langerhans cell histiocytosis (LCH) is a rare inflammatory disorder of myeloid dendritic cells with mutations involving KRAS, BRAF and/or NRAS, and MAP2K1 genes. We describe the case of a 58-year-old female previous smoker with multifocal LCH involving the lungs, pituitary gland and mandibular bone. Initial treatment with 6 cycles of cladribine showed improvement in her extrapulmonary lesions, however, her lung disease progressed and after qualification and assessment tests she underwent uncomplicated double lung transplant surgery and was discharged home. We highlight that in select patients with well managed and controlled extrapulmonary LCH, such an invasive procedure as lung transplant is possible.
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