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: Hemalatha G Rangarajan, Darci Grochowski, Mollie F Mulberry, Gabriela Gheorghe, Bruce M Camitta, Julie-An M Talano
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 59 Issue 1 Pg. 189-90 (Jul 15 2012) ISSN: 1545-5017 [Electronic] United States
• PMID: 21755594 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2011 Wiley Periodicals, Inc.

Chemical References

• Anti-Inflammatory Agents
• Dexamethasone

Topics

• Anti-Inflammatory Agents (administration & dosage)
• Bone Marrow Transplantation
• Central Nervous System Diseases (drug therapy, etiology)
• Dexamethasone (administration & dosage)
• Female
• Humans
• Infant
• Lymphohistiocytosis, Hemophagocytic (complications, therapy)
• Transplantation, Homologous