Prognostic analysis of children diagnosed hemophagocytic lymphohistiocytosis with central nervous system involvement in children

Abstract

Objective To explore the prognostic outcomes of children with hemophagocytic lymphohistiocytosis (HLH) and central nervous system (CNS) involvement. Methods Clinical data of 61 HLH children including CNS symptoms, cranial imaging findings (CT/MRI) and cerebrospinal fluid (CSF) results were collected retrospectively, who were admitted to Hematology Oncology Center of Beijing Children′s Hospital, Capital Medical University from January 2012 to December 2013.The relationship was evaluated between CNS involvement and prognosis by comparing the overall survival (OS) rate. Results In the total 61 cases, median age on diagnosis was 28.1 months, and median follow-up time was 45 months; OS rate was 61.1%(33/54 cases, 7 cases lost follow-up). Thirty-three children (54.1%) were diagnosed as EB virus-HLH, with median age of 48.9 months and OS rate of 69.0% (20/29 cases, 4 cases lost to follow-up), which was higher than other HLHs (52.0%, 13/25 cases), but the difference was not significant(χ2=1.626, P=0.202). There were 24.6% of children with CNS clinical manifestations, and the OS rate was 35.7% (5/14 cases), which was lower than those who had no CNS symptoms (70.0%, 28/40 cases), and the difference was significant (χ2=5.129, P=0.024). There were 61.5% of patients with changed imaging findings, and their OS rate was 54.8% (17/31 cases), which was also lower than those with no changes in CT and/or MRI (87.5%, 14/16 cases), and the difference was significant (P=0.049). While children with abnormal CSF accounted for 19.2% of those who received lumbar puncture, and their OS rate was 40.0% (2/5 cases), which was lower than those with normal CSF (81.0%, 17/21 cases), but the difference was not significant(P=0.101). Any change in CNS symptom, imaging finding or CSF was regarded as CNS involvement.Apart from children who did not have data of CT/MR and/or CSF, the proportion of patients with CNS involvement was 70.4%(38/54 cases), their OS rate was 54.1%(20/37 cases, and 1 case lost follow-up), which was lower than those without CNS impairment (91.7%, 11/12 cases), and the difference was significant (P=0.036). Conclusions Pediatric patients with HLH always have poor prognosis once CNS symptoms or abnormal imaging findings can be founded.CNS involvement is an important risk factor to estimating the patients′ outcome.Cranial MRI and CSF test are necessary for newly diagnosed HLH children, regardless of whether neurological manifestation could be discovered. Key words: Child; Hemophagocytic lymphohistiocytosis; Central nervous system