[Clinical features and therapeutic results in 14 cases of XXXX in childhood].

@entity1 @entity3329 @entity410 @entity8 @entity632 @entity1087 @entity1910 @entity146 @entity5 @entity229 @entity786 @entity2879 @entity828 @entity5642 @entity3043 @entity358 @entity1891 @entity6805 @entity221
Fourteen @entity1 , 4 males and 10 females, with @entity5 ( @entity5 ) were treated between July 1980 and June 1986. None of them had an affected sibling with a similar disorder. @entity221 was the most prominent symptom in all cases. @entity828 was present in 13 cases, @entity3329 , @entity1910 and @entity1087 in 8, @entity358 or pleural effusion on chest X-ray in 8, @entity410 in 6, and @entity8 in 5 out of the 14 cases. @entity5642 ( @entity5642 ) was seen in 13 cases and this occurred within two weeks from onset in 6 cases. @entity3043 , abnormal results of liver function tests, @entity6805 and hypocholesterolemia were common. The diagnosis was made for all 14 cases by characteristic clinical symptoms, signs, and bone marrow findings. In 8 cases, biopsy or autopsy specimens confirmed the diagnosis. Two @entity1 died prior to chemotherapy. Twelve @entity1 were treated with @entity632 , @entity786 , @entity1891 and @entity229 (ACOP). Complete response (CR) was achieved in five @entity1 , and another two @entity1 attained CR after subsequent treatment with other combinations including @entity2879 . These 7 complete responders are now alive and free of disease 11+ to 70+ months (median, 50+ months) from the onset of disease. All partial and non-responders died within 6 months with a median survival of 20 days. Among several clinical features as prognostic indicators, @entity8 , @entity410 , and @entity5642 occurring within 2 weeks were significantly related to poor outcome. Although @entity5 is an @entity146 with a poor prognosis, prompt diagnosis and early treatment with intensive systemic combination chemotherapy should further improve the outcome.
