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Titolo:
LONG-TERM SURVIVORS IN MYELODYSPLASTIC SYNDROMES - A REPORT ON 63 CASES AND COMPARISON WITH SHORT AND INTERMEDIATE SURVIVORS
Autore:
WATTEL E; HECQUET B; GRAHEK D; HEBBAR M; MOREL P; LAI JL; BAUTERS F; FENAUX P;
Indirizzi:
CHU LILLE,SERV MALADIES SANG,1 PL VERDUN F-59037 LILLE FRANCE CHU LILLE,SERV MALADIES SANG,1 PL VERDUN F-59037 LILLE FRANCE CTR OSCAR LAMBRET,SERV BIOSTAT F-59020 LILLE FRANCE CHU LILLE,SERV CYTOGENET F-59037 LILLE FRANCE
Titolo Testata:
Leukemia research
fascicolo: 9, volume: 17, anno: 1993,
pagine: 733 - 739
SICI:
0145-2126(1993)17:9<733:LSIMS->2.0.ZU;2-M
Fonte:
ISI
Lingua:
ENG
Soggetto:
CHRONIC MYELOMONOCYTIC LEUKEMIA; PROGNOSTIC FACTORS; SCORING SYSTEM; FAB CLASSIFICATION; PROPOSALS;
Keywords:
MYELODYSPLASTIC SYNDROMES; LONG TERM SURVIVORS; PROGNOSIS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
24
Recensione:
Indirizzi per estratti:
Citazione:
E. Wattel et al., "LONG-TERM SURVIVORS IN MYELODYSPLASTIC SYNDROMES - A REPORT ON 63 CASES AND COMPARISON WITH SHORT AND INTERMEDIATE SURVIVORS", Leukemia research, 17(9), 1993, pp. 733-739

Abstract

Sixty-three of 373 patients (17%) with de novo myelodysplastic syndrome (MDS) survived more than 5 yr (long survivors). At diagnosis, they usually had no or only moderate cytopenias; 63% of them had marrow blasts <5%; only 1 patient had circulating blasts; 60% had refractory anaemia (RA) or refractory anaemia with ringed sideroblasts (RARS); 19% of the 43 patients who were karyotyped had an abnormal clone, and only 2 patients had complex cytogenetic findings. Only 4 of the 63 patientsprogressed to another FAB type within 5 yr of diagnosis, from RA to refractory anaemia with excess of blasts (RAEB) or chronic myelomonocytic leukaemia (CMML). After 5 yr, 9 patients showed progression (including progression to AML in 5 patients). The other patients remained stable (43 cases) or died from cytopenias or unrelated disorders (11 cases). Except for 3 patients who achieved prolonged complete remission with intensive chemotherapy, and possibly 3 patients who responded to low dose Ara C, prolonged survival seemed to result mainly from the low natural course of the disease. Although, as expected, significant differences for age, cytopenias, circulating and bone marrow blasts, and karyotype were seen between short (<2 yr) and long (>5 yr) very few significant differences were seen between intermediate and long survivors. These 2 subgroups only differed by significantly lower age and higher haemoglobin level in long survivors. Multivariate scoring systems (Bournemouth, Sanz's and Lille scores) proved of relatively limited value in differentiating them. Thus, currently known prognostic factors inMDS are capable of identifying short survivors, but cannot as readilydistinguish patients who will have an intermediate survival from truly long survivors.

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Documento generato il 14/08/20 alle ore 16:45:57