What are Histiocytic Disorders? HistiocyticDisorders Histiocytes
References Histiocytic disorders are a group of disorders arising from an over-production of histiocytic white blood cells called histiocytes. ‘Histiocytosis’, meaning ‘tissue cell’, is a general concept for diseases exemplified by myeloid cells, namely mononuclear phagocytes, that share histological features with macrophages or dendritic cells.
LCH-associated neurodegeneration is a severe complication that can arise with systemic symptoms or several years following an initial LCH diagnosis. LCH-NF shows clinical signs of cerebella dysfunction and abnormal MRI findings in the cerebellum, pons, and brain stem. Positive clinical responses have been demonstrated with chemotherapy and MAPK inhibitors.
Numerous types of histiocytosis have been reviewed in the literature. Overarchingly, they represent a collection of disorders that share a terminal phenotype with features that are congruent with mononuclear phagocytotic cells. LCH, ECD, JXG, and some cases of RDD represent clonal disorders associated with oncogene drivers, which cause aberrant differentiation of myeloid precursors.
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