Analysis of myelodysplastic syndrome patients with deletion of long arm of chromosome 5 followed up by the Czech MDS Working Group. Significance for the diagnostic and prognostic assessment
Authors:
R. Neuwirtová 1; A. Jonášová 1; J. Čermák 2; J. Vondráková 3; M. Šišková 1; I. Hochová 4; V. Vozobulová 5; A. Jungová 5; P. Bělohlávková 6; E. Kadlčková 7; O. Černá 8; J. Libiger 9; A. Smolíková 10; E. Polonyová 11; N. Seifertová 12; K. Michalová 1; J. Březinová 2; D. Mikulenková 2
Authors‘ workplace:
I. interní klinika – klinika hematologie VFN Praha, 2Ústav hematologie a krevní transfuze Praha
1; Hemato-onkologické klinika FN Olomouc, 4OKH FN Motol Praha, 5Hemato-onkologické odd. FN Plzeň
3; OKH FN Hradec Králové, 7HTO Baťova nemocnice Zlín, HO FN Královské Vinohrady Praha
6; OKH Masarykova nemocnice Ústí n. L., HO KlinLab Praha, HO Nemocnice Karlovy Vary
9; OKH Nemocnice České Budějovice, (Statistická spolupráce ing. A. Dohnalová 1. LF Praha)
12
Published in:
Transfuze Hematol. dnes,15, 2009, No. 4, p. 204-209.
Category:
Comprehensive Reports, Original Papers, Case Reports
Overview
There are new important discoveries in the pathogenesis and therapy of MDS patients with del(5q). To be well prepared for rational application of new findings for precise diagnosis and new therapy, clinical and laboratory data of our 68 MDS patients with del(5q) were analyzed. Patients were divided into four groups. A – 5q-syndrome with normal platelet count and B – with thrombocytemia, C – patients who did not fulfill two or more out of six criteria of the 5q-syndrome or had thrombocytopenia, D – patients with further chromosomal aberrations beside del(5q). Sex, age, hematological findings, transfusion dependence and prognostic score (IPSS and WPSS) were evaluated. Survival was calculated in the group of dead patients. AML incidence and time to transformation into AML were also registered. In all 4 groups women prevailed, mostly in the group B. Hypoplastic erythropoiesis predominated in patients with 5q-syndrome and was rare in those of group D. In all groups typical dysplastic megakaryocytes were found. The longest survival was observed in patients of group B – 5q-syndrome with thrombocythemia. The shortest survival we found in dead patients of group D. Nevertheless, living patients having only one further chromosomal aberration did not differ from those of group A. The incidence of AML was lower compared to the percentage set for MDS in general. AML transformation developed at earliest in patients of group D, at latest in group A and was exceptional in group B. To determine the prognosis of patients with del(5q) as reliably as possible we prefer WPSS to IPSS, we recommend to take in consideration the thrombocytopenia and to try to insert the patients into one of above mentioned groups. To determine the prognosis is especially important for patients of group C and D, because in younger patients with unfavorable prognosis stem cells transplantation needs to be considered.
Key words:
myelodysplastic syndrome, del(5q), clinical and laboratory picture, prognostic assessment
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Labels
Haematology Internal medicine Clinical oncologyArticle was published in
Transfusion and Haematology Today
2009 Issue 4
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