Neuroradiologic Imaging Findings in Histiocytosis Syndromes in Children and Young Adults
There are 2 major subtypes of histiocytosis syndrome in children and adults, classified according to their cellular basis: disorders of varied biologic behavior (dendritic cell‐related or macrophage-related) and malignant disorders (monocyte-related, dendritic cell‐related
histiocytic sarcoma, or macrophage-related histiocytic sarcoma). While some neuroradiologic manifestations of histiocytosis syndromes are more closely linked to the underlying pathophysiology, others are less well-defined and remain within a broader differential diagnostic consideration. The
underlying epidemiology, pathophysiology, and known radiologic manifestations are reviewed to aid the neuroradiologist in considering the diagnosis of histiocytosis in the appropriate clinical setting.
Keywords: ADEM = acute disseminated encephalomyelitis; CN = cranial nerve; DI = diabetes insipidus; FDG = fluorodeoxyglucose; FHL = familial hemophagocytic lymphohistiocytosis; HLH = hemophagocytic lymphohistiocytosis; LCH = Langerhans cell histiocytosis; MAHS = malignancy-associated hemophagocytic syndrome; RES = reticuloendothelial system; SHML = sinus histiocytosis with massive lymphadenopathy; TB = tuberculosis; VAHS = virus-associated hemophagocytic syndrome; sHLH = secondary hemophagocytic lymphohistiocytosis
Document Type: Research Article
Publication date: 01 September 2012
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