A population‐based study of 272 children with acute myeloid leukaemia treated on two consecutive protocols with different intensity: best outcome in girls, infants, and children with Down's syndrome

Lie, Sverre O.; Jónmundsson, Guđmundur; Mellander, Lotta; Ma, Siimes; Yssing, M.; Gustafsson, Göran

doi:10.1046/j.1365-2141.1996.d01-1761.x

Cited by 126 publications

(107 citation statements)

References 23 publications

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“…The age distribution appears similar to what is found in MDS and AML without −7/7q−. 1,40 There was a strong male predominance in MDS, whereas girls dominated in AML. A female predominance has previously been reported among children below 2 years of age with AML.…”

Section: Discussionsupporting

confidence: 51%

Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7

et al. 1999

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We reviewed the clinical features, treatment, and outcome of 100 children with myelodysplastic syndrome (MDS), juvenile myelomonocytic leukemia (JMML), and acute myeloid leukemia (AML) associated with complete monosomy 7 (−7) or deletion of the long arm of chromosome 7 (7q−). Patients with therapyinduced disease were excluded. The morphologic diagnoses according to modified FAB criteria were: MDS in 72 (refractory anemia (RA) in 11, RA with excess of blasts (RAEB) in eight, RAEB in transformation (RAEB-T) in 10, JMML in 43), and AML in 28. The median age at presentation was 2.8 years (range 2 months to 15 years), being lowest in JMML (1.1 year). Loss of chromosome 7 as the sole cytogenetic abnormality was observed in 75% of those with MDS compared with 32% of those with AML. Predisposing conditions (including familial MDS/AML) were found in 20%. Three-year survival was 82% in RA, 63% in RAEB, 45% in JMML, 34% in AML, and 8% in RAEB-T. Children with −7 alone had a superior survival than those with other cytogenetic abnormalities: this was solely due to a better survival in MDS (3-year survival 56 vs 24%). The reverse was found in AML (3-year survival 13% in −7 alone vs 44% in other cytogenetic groups). Stable disease for several years was documented in more than half the patients with RA or RAEB. Patients with RA, RAEB or JMML treated with bone marrow transplantation (BMT) without prior chemotherapy had a 3-year survival of 73%. The morphologic diagnosis was the strongest prognostic factor. Only patients with a diagnosis of JMML fitted what has previously been referred to as the monosomy 7 syndrome. Our data give no support to the concept of monosomy 7 as a distinct syndrome.

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Section: Discussionsupporting

confidence: 51%

Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7

et al. 1999

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“…We studied 28 cases of T-ALL (11 adults and 17 children) and occurrence of the BCR/ABL rearrangement is recognized in 20-30% of adult cases of acute lymphoblastic leukemia, in 10 cases of T-CLD (two cases of large granular lymphocytic leukemia, one case of prolymphocytic leukemia, two cases 2-5% of newly diagnosed pediatric cases and in 12% of first relapse pediatric cases. [1][2][3][4][5] In contrast, the presence of the Philof Sézary syndrome, two cases of adult T cell leukemia/lymphoma and three cases of the leukemia phase of adelphia chromosome in T cell diseases is restricted to a few cases of T cell acute lymphoblastic leukemia (T-ALL). 1,[6][7][8] peripheral T cell lymphoma).…”

Section: Lack Of Bcr/abl Rearrangement In Acute and Chronic T Cell Lementioning

confidence: 99%

“…2,3,4 Ara-C and daunorubicin. The basis of enhanced chemotherapy response of DS chilIncreased activity of cystathionine-␤-synthase localized to dren was examined in vitro, by assessing the sensitivity of 21q22.3 6 may enhance Ara-C metabolism by: (1) altering myeloblasts (obtained from newly diagnosed patients with reduced folate pools leading to decreased deoxycytidine tri-AML registered on the POG 9421 protocol) to Ara-C and dauphosphate (dCTP) pools, and release of feedback inhibition on norubicin utilizing the MTT (3-[4,5-dimethyl-thiazol-2-yl]-2,5-deoxycytidine kinase (dCk), the rate limiting phosphorylating diphenyltetrazolium bromide) cytotoxicity assay.…”

mentioning

confidence: 99%

Myeloblasts from Doen syndrome children with acute myeloid leukemia have increased in vitro sensitivity to cytosine arabinoside and daunorubicine

et al. 1997

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“…10,11 Details of the conditioning regimens have been thoroughly recorded elsewhere. 12 In short, children with ALL and lymphoblastic lymphoma (LBL) were conditioned with prednisolone, teniposide, daunorubicin, vincristine, cyclophosphamide, and cytarabine, plus TBI.…”

Section: Patientsmentioning

confidence: 99%

Pulmonary function after autologous bone marrow transplantation in children: a long-term prospective study

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Arvidson

Bratteby

et al. 2003

Bone Marrow Transplant

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Summary:We performed serial pulmonary function tests (PFTs) consisting of spirometry and diffusing capacity in 26 children after BMT. The median follow-up was 10 years. The influence of total body irradiation (TBI) on long-term pulmonary function was of particular interest. In the 20 children who had received TBI, after an initial decrease the PFTs showed recovery, but the mean lung volumes were still significantly decreased 5 years after BMT at 10% below baseline. The proportions of children with restrictive impairment 5 and 10 years after BMT were 20 and 21%, respectively. Only one child was diagnosed with obstructive impairment. The proportions of children with isolated diffusing impairment at 5 and 10 years were 7/20 (35%) and 7/13 (54%), respectively. Six children had received chemotherapy only and showed isolated diffusing impairment as the only long-term sequela in 4/5 and 1/3 at 5 and 10 years. Our main finding was that there was little change in PFTs 1-10 years after BMT. TBI was associated with persistently decreased lung volumes in a proportion of patients, whereas chemotherapy also might have been of importance for the development of impaired gas exchange.

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A population‐based study of 272 children with acute myeloid leukaemia treated on two consecutive protocols with different intensity: best outcome in girls, infants, and children with Down's syndrome

Cited by 126 publications

References 23 publications

Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7

Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7

Myeloblasts from Doen syndrome children with acute myeloid leukemia have increased in vitro sensitivity to cytosine arabinoside and daunorubicine

Pulmonary function after autologous bone marrow transplantation in children: a long-term prospective study

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