Langerhans cell histiocytosis

Abstract

The first major stride toward understanding LCH was taken when ultrastructural studies identified the proliferating cells as part of the Langerhans (dendritic) cell system. Another step forward was the definition of the morphologic, immunohistochemical, and clinical criteria needed for the diagnosis of LCH. Meanwhile, modern imaging studies have disclosed lesions that were not previously visible, especially those in the brain and the pituitary gland. These advantages have had a major impact on clinical management by making it possible to compare data from different institutions and to centralize coherent clinical and therapeutic data. Moreover, the agreement concerning diagnostic criteria provides a solid foundation for current clinical trials and for laboratory research regarding the possible roles of the immune system, clonality, and cytokines in the etiology of LCH.