Prognosis Of The Central Nervous System Involvement In Patients With Hemophagocytic Lymphohistiocytosis

Objectives: To investigated the characteristics and prognostic factor of central nervous system(CNS)involvement in patients with hemophagocytic lymphohistiocytosis(HLH).Methods:From January 2006 to October 2015 152 patients with HLH diagnosed and treated in the Blood Tumor center of Children's Hospital affiliated with Chongqing Medical University,88 patients had CNS involvement,analyse their clinical information,Survival rates were analysed using the Kaplan–Meier life table method,using univariate and multivariate COX regression model analyse the risk factors of prognosis.Results:1.152 patients diagonosed HLH and treated in our Hospital from January 2006 to October 2015,of which 88 patients had CNS involvement.These 88 patients,73 had EBV infection,2CMV infection,8bacterial infection;The remaining 5 patients did not find a definite etiology.2.59 patients have neurological symptoms,including seizures,altered consciousness,paralysis,mental and behavior disorder,ataxia,neck stiffness,opisthotonus.20 patients CSF analysis consistently showed a lymphocytic pleocytosis and elevated protein.42 patients had abnormal brain imaging results,including deepen or widening of brain Sulci,atrophy,periventricular white matter abrnomal,hemorrhages,calcification,and so on.3.36 patients survived well,3 patients lost to follow-up,49 dead,1 survival patient had epilepsy.The 3-year overall survival rate of all patients was 44%,Median survival time was 4.5 month.4.abnormal CSF and didn't intrathecal injections bears a significant,independent adverse prognostic value(P<0.05).Conclusions:1.CNS involvement in HLH has a high frequency and poor prognosis,few patients remains neurologic sequelae.2.abnormal CSF relate to poor prognosis,positive systemic chemotherapy and intrathecal injections is effective in children with secondary HLH.