Long-term results of AIEOP-8805 protocol for XXXX of childhood.

@entity1 @entity6220 @entity137 @entity5 @entity2225 @entity2109 @entity1778 @entity8625 @entity236
@entity8625 ( @entity8625 ) is a rare form of @entity1778 characterized by a very high-proliferation index, rapid clinical progression, and a high frequency of central nervous system (CNS) involvement. Commonly, it is treated in the clinical trials for @entity2109 , of which it represents the leukemic counterpart. PROCEDURE: @entity1 with @entity8625 diagnosed between 1988 and 1999 were enrolled in the AIEOP-8805 protocol. Treatment included six high-dose chemotherapy courses. No prophylactic CNS irradiation was administered. RESULTS: Sixty-five consecutive @entity1 were enrolled in the study. L3 morphology was observed in 57 of 65 @entity1 (88%). Twenty-five @entity1 (38%) had @entity5 mass in addition to massive @entity236 ; 11 @entity1 (17%) had @entity6220 at diagnosis. Sixty-two @entity1 obtained complete morphological remission of which 13 suffered a relapse, including 3 with initial CNS involvement. Ten-year overall survival and event-free survival were 77% and 75%, respectively. Neither relevant long-term @entity137 nor second @entity5 were observed. CONCLUSIONS: The AIEOP-8805 confirmed that short high-dose chemotherapy is highly effective for the treatment of @entity8625 without significant long-term adverse sequelae. Therapy modifications to reduce relapse rate, such as the use of anti- @entity2225 monoclonal antibody and more effective CNS treatment, are being tested.
