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Treatment of recurrent CNS disease post‐bone marrow transplant in familial HLH
Author(s) -
Rangarajan Hemalatha G.,
Grochowski Darci,
Mulberry Mollie F.,
Gheorghe Gabriela,
Camitta Bruce M.,
Talano JulieAn M.
Publication year - 2011
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.23248
Subject(s) - medicine , hemophagocytic lymphohistiocytosis , blood cancer , bone marrow , disease , dexamethasone , pediatrics , cancer
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. Pediatr Blood Cancer 2012; 59: 189–190. © 2011 Wiley Periodicals, Inc.