Premium
Treatment of neurodegenerative CNS disease in Langerhans cell histiocytosis with a combination of intravenous immunoglobulin and chemotherapy
Author(s) -
Imashuku Shinsaku,
Okazaki Nagisa Amamoto,
Nakayama Masahiko,
Fujita Naoto,
Fukuyama Tetsuhiro,
Koike Kenichi,
Minato Toshinori,
Kobayashi Ryoji,
Morimoto Akira
Publication year - 2008
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.21259
Subject(s) - medicine , langerhans cell histiocytosis , chemotherapy , methotrexate , blood cancer , vinblastine , ataxia , gastroenterology , disease , cancer , psychiatry
Background In rare cases, patients with Langerhans cell histiocytosis (LCH) develop neurodegenerative CNS disease (ND‐CNS‐LCH). Management of ND‐CNS‐LCH has not been established. Methods We treated five pediatric patients with a combination of intravenous immunoglobulin (IVIG) and chemotherapy (steroid ± vinblastine ± 6‐mercaptopurine ± methotrexate). Prior to the therapy, three of the five patients had cerebellar ataxia while the remaining two had abnormal MRI findings without apparent neurological deficits. IVIG was given monthly or twice monthly at the dosage of 250–400 mg/kg/dose. Results The four patients administered more than 23 doses of IVIG and chemotherapy remained in a stable condition and did not show significant progression signs in neurological deficits or brain MRI findings during the 30‐month follow‐up period (median; range: 19+ to 38+) following the initiation of therapy for ND‐CNS‐LCH. Conclusion The IVIG‐containing treatment may be promising for ND‐CNS‐LCH; however, its effectiveness remains to be further tested in more patients as well as in a randomized trial. Pediatr Blood Cancer 2008;50:308–311. © 2007 Wiley‐Liss, Inc.