Successful treatment of adult onset Langerhans cell histiocytosis with multi-drug combination therapy.

Adult onset Langerhans cell histiocytosis (LCH) is a rare disorder. Its clinical features have been well described in children, however remain poorly defined in adults. Optimal treatment strategy is still under debate. We have encountered two cases of adult onset LCH, which obtained a durable disease control by combination chemotherapy using prednisone, vinblastine and 6-mercaptopurine. Herein, we report their clinical features together with a review of the current literature.

[1]  F. Follath,et al.  Langerhans'-cell histiocytosis in adults. , 1997, Medical and pediatric oncology.

[2]  W. High,et al.  Langerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte Society , 2008, Pediatric dermatology.

[3]  J. Birch,et al.  Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998 , 2007, Pediatric blood & cancer.

[4]  A. Saven,et al.  Cladribine activity in adult langerhans-cell histiocytosis. , 1999, Blood.

[5]  U. Pötschger,et al.  Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. , 2008, Blood.

[6]  E. von Stebut,et al.  Successful treatment of adult multisystemic Langerhans cell histiocytosis with psoralen-UV-A, prednisolone, mercaptopurine, and vinblastine. , 2008, Archives of dermatology.

[7]  S. Pileri,et al.  MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: experience on seven patients. , 2010, Annals of oncology : official journal of the European Society for Medical Oncology.

[8]  B P Mullan,et al.  Langerhans cell histiocytosis , 1999, Cancer.

[9]  J. Michaelis,et al.  A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. , 2001, The Journal of pediatrics.

[10]  K. Matthay,et al.  Disease course and late sequelae of Langerhans' cell histiocytosis: 25-year experience at the University of California, San Francisco. , 1996, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[11]  P. Monnier,et al.  Multisystem Langerhans’ cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature , 2004, European Archives of Oto-Rhino-Laryngology and Head & Neck.

[12]  Egeler Rm.  [Langerhans-cell histiocytosis]. , 1993 .

[13]  C. Sucker,et al.  Adult Langerhans cell histiocytosis , 2006, European journal of haematology.

[14]  H. Gadner,et al.  Current therapy for Langerhans cell histiocytosis. , 1998, Hematology/oncology clinics of North America.

[15]  A. Chu,et al.  Treatment of adult Langerhans cell histiocytosis with etoposide. , 1992, Journal of the American Academy of Dermatology.

[16]  U. Pötschger,et al.  Response to initial treatment of multisystem Langerhans cell histiocytosis: an important prognostic indicator. , 2002, Medical and pediatric oncology.

[17]  J. Emile,et al.  Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. , 2003, European journal of cancer.

[18]  M. Aricò.  Langerhans' cell histiocytosis in adults: another orphan disease? , 1999, Haematologica.