Highlights of the Management of Adult Histiocytic Disorders: Langerhans Cell Histiocytosis, Erdheim-Chester Disease, Rosai-Dorfman Disease, and Hemophagocytic Lymphohistiocytosis
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Elsevier Inc.
Abstract
Histiocytic disorders are an exceptionally rare group of diseases with diverse manifestations and a paucity of approved treatments, thereby leading to various challenges in their diagnosis and management. With the discovery of novel molecular targets and the incorporation of targeted agents in the management of various adult histiocytic disorders, their management has become increasingly complex. In an attempt to improve the understanding of the clinical features and management of common adult histiocytic disorders (Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease, and hemophagocytic lymphohistiocytosis), we created this document based on existing literature and expert opinion. © 2020 Elsevier Inc.
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Braf, Covid-19, Mek, Targeted, Treatment, Adult, Drug therapy, combination, Erdheim-chester disease, Histiocytosis, langerhans-cell, Histiocytosis, sinus, Humans, Lymphohistiocytosis, hemophagocytic, Treatment outcome, Alemtuzumab, Alpha interferon, Anakinra, Azathioprine, B raf kinase inhibitor, Bleomycin, Blinatumomab, Cladribine, Clofarabine, Cobimetinib, Corticosteroid, Cyclophosphamide, Cyclosporine, Desmopressin, Dexamethasone, Doxorubicin, Emapalumab, Etoposide, Immune checkpoint inhibitor, Immunoglobulin, Lenalidomide, Mercaptopurine, Methotrexate, Mitogen activated protein kinase kinase inhibitor, Prednisolone, Prednisone, Rapamycin, Rituximab, Ruxolitinib, Steroid, Thalidomide, Tocilizumab, Trametinib, Vemurafenib, Vinblastine, Vincristine, Allogeneic hematopoietic stem cell transplantation, Autologous hematopoietic stem cell transplantation, Clinical feature, Clinical outcome, Coronavirus disease 2019, Diagnostic procedure, Erdheim chester disease, Gene mutation, Hemophagocytic syndrome, Human, Immunotherapy, Infection, Langerhans cell histiocytosis, Macrophage activation syndrome, Malignant neoplasm, Myeloablative conditioning, Pregnancy, Reduced intensity conditioning, Review, Sinus histiocytosis, Smoking cessation, Treatment response, Combination drug therapy