Are we justified to classify patients with two unrelated clones with del(5q) and trisomy 8 as a subgroup of myelodysplastic syndrome of the type of 5q- syndrome?
Authors:
R. Neuwirtová 1; Z. Zemanová 2; J. Březinová 3; M. Beličková 3; P. Dvořák 4; A. Oltová 7; A. Jonášová 1; J. Čermák 3; D. Šponerová 3; J. Ullrichová 5; Z. Mašková 6; L. Červinek 7; Y. Smělíková 8; V. Vozobulová 9; E. Polonyová 10; M. Svoboda 11; K. Michalová 2
Authors‘ workplace:
I. interní klinika – klinika hematologie, Všeobecná fakultní nemocnice (VFN), 1. lékařská fakulta Univerzity Karlovy Praha
1; Centrum nádorové cytogenetiky, ÚLBLD, VFN Praha
2; Ústav hematologie a krevní transfuze Praha
3; Ústav lékařské genetiky, FN Plzeň
4; Oddělení klinické hematologie (OKH), Masarykova nemocnice Ústí nad Labem
5; OKH, Fakultní Thomayerova nemocnice Praha
6; Interní hematoonkologická klinika, FN Brno
7; OKH, Nemocnice Kroměříž
8; I. interní klinika, FN Plzeň
9; OKH, Nemocnice Karlovy Vary
10; OKH, Nemocnice Jindřichův Hradec
11
Published in:
Transfuze Hematol. dnes,20, 2014, No. 1, p. 25-31.
Category:
Comprehensive Reports, Original Papers, Case Reports
Overview
Among the 106 patients with myelodysplastic syndrome (MDS) of the type 5q minus syndrome, we found 11 patients who comply with the criteria of this syndrome. In addition to the del(5q) clone they were found to have the unrelated, independent trisomy 8 clone. All these patients were female. In ten patients, the del(5q) clone size was significantly larger than the +8 clone. The patients’ survival is long and no patient transformed into AML. Only one patient died; her +8 clone size was twice that of her del(5q) clone. Two of our patients are successfully being treated with lenalidomide, a drug with high efficiency in 5q- syndrome. Based on the clinical observation of our patients with both unrelated clones del(5q) and +8, we believe we can consider these patients as the subgroup of the 5q- syndrome and to expect the same favorable prognosis and similar efficacy of treatment as in the typical 5q- syndrome. Moreover, our results should remind hematologists that, when dealing with biclonality in MDS, it is necessary to assess the clones’ sizes, with the assumption that the larger clone will have a predominant effect on the prognosis. In patiens with clinical and laboratory symptoms of 5q- syndrome and trisomy 8 under “cut off value“ repeated cytogenetic examination is indicated.
Key words:
5q- syndrome, biclonality, del(5q)-trisomy 8
Sources
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Haematology Internal medicine Clinical oncologyArticle was published in
Transfusion and Haematology Today
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