Treatment of neurodegenerative CNS disease in Langerhans cell histiocytosis with a combination of intravenous immunoglobulin and chemotherapy.
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.


CONCLUSIONS
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.
Imashuku
Shinsaku
S
Division of Pediatrics, Takasago-seibu Hospital, Takasago, Japan. shinim95@mbox.kyoto-inet.or.jp
Okazaki
Nagisa ' Amamoto '
N'
Nakayama
Masahiko
M
Fujita
Naoto
N
Fukuyama
Tetsuhiro
T
Koike
Kenichi
K
Minato
Toshinori
T
Kobayashi
Ryoji
R
Morimoto
Akira
A
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D019636:Neurodegenerative Diseases / Q000188:drug therapy* / Q000473:pathology; D014747:Vinblastine / Q000008:administration & dosage
D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D014747:Vinblastine; D015122:Mercaptopurine; D008727:Methotrexate