hematology and oncology
Langerhans Cell Histiocytosis
Treatment of Langerhans Cell Histiocytosis
Supportive care Sometimes hormone replacement therapy for hypopituitarism, most commonly diabetes insipidus Chemotherapy for multisystem involvement, single system multifocal involvement, and involvement in certain sites such as skull-based lesions Sometimes surgery, corticosteroid injection, or rarely, radiation therapy (usually for unifocal bone involvement) General supportive care is essential and may include scrupulous hygiene to limit ear, cutaneous, and dental lesions. Debridement or resection of severely affected gingival tissue limits oral involvement. Seborrhea-like dermatitis of the scalp may diminish with use of aselenium-based shampoo twice a week. If shampooing is ineffective, topical corticosteroids are used in small amounts and briefly in small areas. Patients with systemic disease are monitored for potential chronic disabilities, such as cosmetic or functional orthopedic and cutaneous disorders and neurologic lesions as well as for psychologic problems that may require psychosocial support. Many patients require hormone replacement for diabetes insipidus or other manifestations of hypopituitarism. Chemotherapy is indicated for patients with multisystem involvement, single system multifocal involvement, and disease in certain sites, such as skull lesions (including zygomatic, orbital, sphenoid, temporal, and ethmoid bones— Treatment references ). Protocols sponsored by the Local surgery, corticosteroid injection, curettage, or rarely, radiation therapy is used for disease involving a single bone. These treatments should be done by specialists experienced in treating Langerhans cell histiocytosis. Easily accessible lesions in noncritical locations undergo surgical curettage. Surgery should be avoided when it may result in significant cosmetic deformities, orthopedic deformities, or loss of function. Historically, radiation therapy was sometimes given to patients at risk of skeletal deformity, vision loss secondary to proptosis, pathologic fractures, vertebral collapse, or spinal cord injury or to patients with severe pain. However, with the use of chemotherapy and targeted drugs, it is rarely necessary. Patients with Langerhans cell histiocytosis that progresses despite standard therapy usually respond to more aggressive chemotherapy. Patients who do not respond to salvage chemotherapy may undergo reduced-intensity Hematopoietic Stem Cell Transplantation , experimental chemotherapy, or immunosuppressive or other immunomodulatory therapy. Patients with 1.Minkov M, Grois N, McClain K, et al: Langerhans cell histiocytosis: Histocyte Society evaluation and treatment guidelines. April 2009. 2.Haupt R, Minkov M, Astigarraga I, et al: Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years.Pediatr Blood Cancer60(2):175–184, 2013. doi: 10.1002/pbc.24367 3.Gadner H, Minkov M, Grois N, et al: Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis.Blood121(25):5006–5014, 2013. doi: 10.1182/blood-2012-09-455774 4.Suh JK, Kang S, Kim H, et al: Recent advances in the understanding of the molecular pathogenesis and targeted therapy options in Langerhans cell histiocytosis.Blood Res56(S1):S65–S69, 2021. doi: 10.5045/br.2021.2021013 