title: Treatment of recurrent CNS disease post-bone marrow transplant in familial HLH.
abstract: CNS involvement in Hemophagocytic Lymphohistiocytosis (HLH) has been reported in 63-73% of children at diagnosis [Haddad et al. (1997); Blood 89: 794-800; Horne et al. (2008); Br J Haematol 140: 327-335]. Patients can present with neurologic symptoms, abnormal CSF cytology, abnormal neuro-imaging, or a combination of these findings. CNS involvement is usually associated with a poor prognosis and increased mortality. The 3 year overall survival is 44% in patients with CNS involvement compared to 67% in patients without CNS involvement at diagnosis [Horne et al. (2008); Br J Haematol 140: 327-335]. We describe a treatment strategy employing systemic dexamethasone to control CNS disease in a patient with familial HLH and persistent CNS disease post Bone Marrow Transplant.
authors.affiliation: Department of Pediatrics, Division of Hematology/Oncology/Blood and Marrow Transplant, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA. hrangara@mcw.edu
authors.forename: Hemalatha G
authors.initials: HG
authors.lastname: Rangarajan
authors.forename: Darci
authors.initials: D
authors.lastname: Grochowski
authors.forename: Mollie F
authors.initials: MF
authors.lastname: Mulberry
authors.forename: Gabriela
authors.initials: G
authors.lastname: Gheorghe
authors.forename: Bruce M
authors.initials: BM
authors.lastname: Camitta
authors.forename: Julie-An M
authors.initials: JA
authors.lastname: Talano
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D016026:Bone Marrow Transplantation; D002493:Central Nervous System Diseases / Q000188:drug therapy / Q000209:etiology; D003907:Dexamethasone / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000628:therapy; D014184:Transplantation, Homologous
chemical_list: D000893:Anti-Inflammatory Agents; D003907:Dexamethasone