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 ofintravenous 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. (c) 2007 Wiley-Liss, Inc.
RCT Entities:
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. (c) 2007 Wiley-Liss, Inc.
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Authors: Kenneth L McClain; Jennifer Picarsic; Rikhia Chakraborty; Daniel Zinn; Howard Lin; Harshal Abhyankar; Brooks Scull; Albert Shih; Karen Phaik Har Lim; Olive Eckstein; Joseph Lubega; Tricia L Peters; Walter Olea; Thomas Burke; Nabil Ahmed; M John Hicks; Brandon Tran; Jeremy Jones; Robert Dauser; Michael Jeng; Robert Baiocchi; Deborah Schiff; Stanton Goldman; Kenneth M Heym; Harry Wilson; Benjamin Carcamo; Ashish Kumar; Carlos Rodriguez-Galindo; Nicholas S Whipple; Patrick Campbell; Geoffrey Murdoch; Julia Kofler; Simon Heales; Marian Malone; Randy Woltjer; Joseph F Quinn; Paul Orchard; Michael C Kruer; Ronald Jaffe; Markus G Manz; Sergio A Lira; D Williams Parsons; Miriam Merad; Tsz-Kwong Man; Carl E Allen Journal: Cancer Date: 2018-04-06 Impact factor: 6.860
Authors: Michael Girschikofsky; Maurizio Arico; Diego Castillo; Anthony Chu; Claus Doberauer; Joachim Fichter; Julien Haroche; Gregory A Kaltsas; Polyzois Makras; Angelo V Marzano; Mathilde de Menthon; Oliver Micke; Emanuela Passoni; Heinrich M Seegenschmiedt; Abdellatif Tazi; Kenneth L McClain Journal: Orphanet J Rare Dis Date: 2013-05-14 Impact factor: 4.123