A. Ceci et al., RECENT AND NEW STUDIES IN LANGERHANS CELL HISTIOCYTOSIS, International journal of pediatric hematology/oncology, 2(4), 1995, pp. 291-298
Since 1985, when the International Histiocyte Society was founded, our
knowledge of Langerhans cell histiocytosis, previously called histioc
ytosis (HX), has dramatically increased. The Histiocyte Society has en
couraged experts to work together to overcome principal controversies.
Because it is a rare nonmalignant disease, international studies are
particularly indicated to collect as many cases as possible, to compar
e different experience, and to adopt common methods of evaluation of t
he results. The main objectives of the Histiocyte Society-LCH Study ar
e as follows: (1) to verify in a large population of patients the crit
eria proposed by the Histiocyte Society for diagnosis and treatment of
Langerhans cell histiocytosis; (2) to promote in situ investigations
concerning the histopathogenesis of the disease and to identify new di
agnostic and prognostic markers; (3) to design a common clinical stagi
ng and follow-up system; (4) to verify the efficacy of different drugs
(i.e., VP-16 and vinblastine) commonly used in the treatment of the d
isease; and (5) to identify subsets of Langerhans cell histiocytosis p
atients who need conservative or alternative treatments.