AIMS: To examine the clinical and biological features of acute lymphoblastic leukaemia in children with Down's syndrome (DS), to compare their survival with other children, and to determine if entry to trials and survival has improved. METHODS: Examination of presenting features and response to treatment in patients treated in two consecutive national trials, MRC UKALL X and XI. RESULTS: The proportion of children with DS was significantly higher in UKALL XI (1.9%) than UKALL X (0.9%). Children with DS tended to be under 10 years and to have the common ALL subtype. Cytogenetic analysis showed that favourable features, such as high hyperdiploidy and t(12;21) were less frequent but also that there was a lack of translocations associated with a poor prognosis. Children with DS showed no increase in risk of relapse at any site but their survival and event free survival were inferior to other children. These results were caused by an increased number of infective deaths during remission (11% compared to 2%). At five years overall survival was 73% in DS children compared with 82% in other children; event free survival was 53% compared to 63% in non-DS children. CONCLUSIONS: Entry of children with DS to national trials has increased and survival has improved. However they remain at risk of relapse and also of treatment related mortality. These findings emphasise the need for both intensive chemotherapy and optimal supportive care.
AIMS: To examine the clinical and biological features of acute lymphoblastic leukaemia in children with Down's syndrome (DS), to compare their survival with other children, and to determine if entry to trials and survival has improved. METHODS: Examination of presenting features and response to treatment in patients treated in two consecutive national trials, MRC UKALL X and XI. RESULTS: The proportion of children with DS was significantly higher in UKALL XI (1.9%) than UKALL X (0.9%). Children with DS tended to be under 10 years and to have the common ALL subtype. Cytogenetic analysis showed that favourable features, such as high hyperdiploidy and t(12;21) were less frequent but also that there was a lack of translocations associated with a poor prognosis. Children with DS showed no increase in risk of relapse at any site but their survival and event free survival were inferior to other children. These results were caused by an increased number of infective deaths during remission (11% compared to 2%). At five years overall survival was 73% in DS children compared with 82% in other children; event free survival was 53% compared to 63% in non-DS children. CONCLUSIONS: Entry of children with DS to national trials has increased and survival has improved. However they remain at risk of relapse and also of treatment related mortality. These findings emphasise the need for both intensive chemotherapy and optimal supportive care.
Authors: D R Hargrave; I I Hann; S M Richards; F G Hill; J S Lilleyman; S Kinsey; C C Bailey; J M Chessells; C Mitchell; O B Eden Journal: Br J Haematol Date: 2001-02 Impact factor: 6.998
Authors: M L Garré; M V Relling; D Kalwinsky; R Dodge; W R Crom; M Abromowitch; C H Pui; W E Evans Journal: J Pediatr Date: 1987-10 Impact factor: 4.406
Authors: A H Ragab; A Abdel-Mageed; J J Shuster; L S Frankel; J Pullen; J van Eys; M P Sullivan; J Boyett; M Borowitz; W M Crist Journal: Cancer Date: 1991-02-15 Impact factor: 6.860
Authors: R Peto; M C Pike; P Armitage; N E Breslow; D R Cox; S V Howard; N Mantel; K McPherson; J Peto; P G Smith Journal: Br J Cancer Date: 1977-01 Impact factor: 7.640
Authors: Kelly W Maloney; William L Carroll; Andrew J Carroll; Meenakshi Devidas; Michael J Borowitz; Paul L Martin; Jeanette Pullen; James A Whitlock; Cheryl L Willman; Naomi J Winick; Bruce M Camitta; Stephen P Hunger Journal: Blood Date: 2010-05-04 Impact factor: 22.113
Authors: Elizabeth G Salazar; Yimei Li; Brian T Fisher; Susan R Rheingold; Julie Fitzgerald; Alix E Seif; Yuan-Shung Huang; Rochelle Bagatell; Richard Aplenc Journal: Br J Haematol Date: 2016-05-10 Impact factor: 6.998
Authors: Yousif Matloub; Karen R Rabin; Lingyun Ji; Meenakshi Devidas; Johann Hitzler; Xinxin Xu; Bruce C Bostrom; Linda C Stork; Naomi Winick; Julie M Gastier-Foster; Nyla A Heerema; Eileen Stonerock; William L Carroll; Stephen P Hunger; Paul S Gaynon Journal: Blood Adv Date: 2019-06-11