title: Cognitive and psychosocial function post hematopoietic stem cell transplantation in children with hemophagocytic lymphohistiocytosis.
abstract: OBJECTIVE
  This study investigated the cognitive and psychosocial outcomes in childhood survivors of hemophagocytic lymphohistiocytosis after hematopoietic stem cell transplantation.
  
  
  METHODS
  Twenty-one children were assessed on standardized measures of cognitive and psychosocial functioning and compared with an unaffected sibling control group (n = 14). Parent and teacher reports were obtained to provide additional information.
  
  
  RESULTS
  The average full-scale intelligence quotient for the patient cohort was 81 (95% CI, 72-90), which was significantly lower than both the population average of 100 (P = .001) and the average for the unaffected sibling control group (99.2, P = .002). Fifty-six percent of school-aged children were receiving additional support at school, with the majority needing high levels of support. These children also experienced significant psychosocial difficulties. Lower socioeconomic status was associated with poorer cognitive outcomes, but age at transplantation, time to transplantation, type of conditioning, and presence of mixed chimerism were not. Ten (48%) of 21 children had evidence of neurologic involvement at diagnosis, but surprisingly, this was not significantly associated with adverse neurologic outcomes, and some children who did not have any apparent neurologic involvement at diagnosis had severe learning difficulties at follow-up.
  
  
  CONCLUSIONS
  In summary, childhood survivors of hemophagocytic lymphohistiocytosis are at risk of long-term cognitive and psychosocial difficulties. Prospective and systematic long-term follow-up of these patients is essential for early identification and effective management of these problems.
authors.affiliation: Research Department of Clinical, Educational and Health Psychology, University College London, London, United Kingdom.
authors.forename: Jessica
authors.initials: J
authors.lastname: Jackson
authors.forename: Penny
authors.initials: P
authors.lastname: Titman
authors.forename: Stephen
authors.initials: S
authors.lastname: Butler
authors.forename: Kathryn
authors.initials: K
authors.lastname: Bond
authors.forename: Anupama
authors.initials: A
authors.lastname: Rao
authors.forename: Paul
authors.initials: P
authors.lastname: Veys
authors.forename: Robert
authors.initials: R
authors.lastname: Chiesa
authors.forename: Alison
authors.initials: A
authors.lastname: Leiper
authors.forename: Lynne
authors.initials: L
authors.lastname: Riley
authors.forename: Kimberly
authors.initials: K
authors.lastname: Gilmour
authors.forename: Persis
authors.initials: P
authors.lastname: Amrolia
authors.forename: Kanchan
authors.initials: K
authors.lastname: Rao
mesh_terms: D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000453:epidemiology*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects* / Q000401:mortality; D006801:Humans; D007223:Infant; D007859:Learning Disabilities / Q000453:epidemiology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000401:mortality / Q000523:psychology* / Q000628:therapy*; D008297:Male; D011446:Prospective Studies; D011584:Psychology; D011795:Surveys and Questionnaires; D017741:Survivors; D016896:Treatment Outcome
keywords: CHS; CNS; CSF; Central nervous system; Cerebrospinal fluid; Chediak-Higashi syndrome; FSIQ; Full-scale intelligence quotient; HLH; HSCT; Hematopoietic stem cell transplantation; Hemophagocytic lymphohistiocytosis; IQ; Intelligence quotient; MRI; Magnetic resonance imaging; SCQ; SDQ; SES; Social Communication Questionnaire; Socioeconomic status; Strengths and Difficulties Questionnaire; UK; United Kingdom; children; cognitive; hematopoietic stem cell transplantation; psychosocial