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Current Treatment for Central Nervous System Involvement in Children with Hemophagocytic Lymphohistiocytosis

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Author:
No author available
Journal Title:
Chinese Medical Journal
Issue:
15
DOI:
10.4103/0366-6999.237413
Key Word:
Central Nervous System;Children;Hemophagocytic Lymphohistiocytosis;Involvement

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a severe life-threatening clinical syndrome with hyperinflammation rather than an isolated disease.It is classified as primary HLH and secondary HLH.Primary HLH is associated with HLH-related gene mutations,whereas secondary HLH occurs after severe infections,tumors,connective tissue diseases,etc.[1] Regardless of primary or secondary HLH,30-73% of patients have central nervous system (CNS) involvement.[2] Among adults with HLH in China,38.4% of patients have neurological symptoms at the onset,and there are up to 89.6% with neurological symptoms during the entire course of the disease.[3] However,it has not been clearly reported about the CNS involvement in children with HLH.

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