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Treatment of recurrent CNS disease post-bone marrow transplant in familial HLH. Pediatr Blood Cancer 2012 Jul 15;59(1):189-90

Date

07/15/2011

Pubmed ID

21755594

DOI

10.1002/pbc.23248

Scopus ID

2-s2.0-84861218979   2 Citations

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.

Author List

Rangarajan HG, Grochowski D, Mulberry MF, Gheorghe G, Camitta BM, Talano JA

Authors

Bruce M. Camitta MD Clinical Professor in the Medicine department at Medical College of Wisconsin
Julie-An M. Talano MD Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Anti-Inflammatory Agents
Bone Marrow Transplantation
Central Nervous System Diseases
Dexamethasone
Female
Humans
Infant
Lymphohistiocytosis, Hemophagocytic
Transplantation, Homologous
jenkins-FCD Prod-409 d1e206b0be345926047b0d9c353c78a4cce4058b