摘要
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.
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.
作者
Hu-Yong Zheng,
Hu-Yong Zheng(Beijing Key Laboratory of Pediatric Hematology Oncology,National Key Discipline of Pediatrics(Capital Medical University;Key Laboratory of Major Diseases in Children,Ministry of Education,Hematology Oncology Center,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,Chin)