RARE CYTOGENETIC ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES

The karyotype represents one of the main cornerstones for the International Prognostic Scoring System (IPSS) and the revised IPSS-R (IPSS-R) that are most widely used for prognostication in patients with myelodysplastic syndromes (MDS). The most frequent cytogenetic abnormalities in MDS, i.e. del(5q...

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Main Authors: Julie Schanz, Friederike Braulke, Detlef Haase
Format: Article
Language:English
Published: PAGEPress Publications 2015-04-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
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Online Access:http://www.mjhid.org/index.php/mjhid/article/view/2262
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author Julie Schanz
Friederike Braulke
Detlef Haase
author_facet Julie Schanz
Friederike Braulke
Detlef Haase
author_sort Julie Schanz
collection DOAJ
description The karyotype represents one of the main cornerstones for the International Prognostic Scoring System (IPSS) and the revised IPSS-R (IPSS-R) that are most widely used for prognostication in patients with myelodysplastic syndromes (MDS). The most frequent cytogenetic abnormalities in MDS, i.e. del(5q), -7/del(7q), +8, complex karyotypes, or –Y have been extensively explored for their prognostic impact. The IPSS-R considers also some less frequent abnormalities such as del(11q), isochromosome 17, +19, or 3q abnormalities. However, more than 600 different cytogenetic categories had been identified in a previous MDS study. This review aims to focus interest on selected rare cytogenetic abnormalities in patients with MDS. Examples are numerical gains of the chromosomes 11 (indicating rapid progression), of chromosome 14 or 14q (prognostically intermediate to favorable), -X (in females, with an intermediate prognosis), or numerical abnormalities of chromosome 21. Structural abnormalities are also considered, e.g. del(13q) that is associated with bone marrow failure syndromes and favorable response to immunosuppressive therapy. These and other rare cytogenetic abnormalities should be integrated into existing prognostication systems such as the IPSS-R. However, due to the very low number of cases, this is clearly dependent on international collaboration. Hopefully, this article will help to inaugurate this process.
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spelling doaj-art-92ff6fe0e6f34b76ba35909bb0107b782025-01-02T06:40:03ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062015-04-0171e2015034e201503410.4084/mjhid.2015.0341560RARE CYTOGENETIC ABNORMALITIES IN MYELODYSPLASTIC SYNDROMESJulie SchanzFriederike BraulkeDetlef HaaseThe karyotype represents one of the main cornerstones for the International Prognostic Scoring System (IPSS) and the revised IPSS-R (IPSS-R) that are most widely used for prognostication in patients with myelodysplastic syndromes (MDS). The most frequent cytogenetic abnormalities in MDS, i.e. del(5q), -7/del(7q), +8, complex karyotypes, or –Y have been extensively explored for their prognostic impact. The IPSS-R considers also some less frequent abnormalities such as del(11q), isochromosome 17, +19, or 3q abnormalities. However, more than 600 different cytogenetic categories had been identified in a previous MDS study. This review aims to focus interest on selected rare cytogenetic abnormalities in patients with MDS. Examples are numerical gains of the chromosomes 11 (indicating rapid progression), of chromosome 14 or 14q (prognostically intermediate to favorable), -X (in females, with an intermediate prognosis), or numerical abnormalities of chromosome 21. Structural abnormalities are also considered, e.g. del(13q) that is associated with bone marrow failure syndromes and favorable response to immunosuppressive therapy. These and other rare cytogenetic abnormalities should be integrated into existing prognostication systems such as the IPSS-R. However, due to the very low number of cases, this is clearly dependent on international collaboration. Hopefully, this article will help to inaugurate this process.http://www.mjhid.org/index.php/mjhid/article/view/2262Myelodysplastic Syndrome, Cytogenetics, Rare Disorders
spellingShingle Julie Schanz
Friederike Braulke
Detlef Haase
RARE CYTOGENETIC ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES
Mediterranean Journal of Hematology and Infectious Diseases
Myelodysplastic Syndrome, Cytogenetics, Rare Disorders
title RARE CYTOGENETIC ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES
title_full RARE CYTOGENETIC ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES
title_fullStr RARE CYTOGENETIC ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES
title_full_unstemmed RARE CYTOGENETIC ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES
title_short RARE CYTOGENETIC ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES
title_sort rare cytogenetic abnormalities in myelodysplastic syndromes
topic Myelodysplastic Syndrome, Cytogenetics, Rare Disorders
url http://www.mjhid.org/index.php/mjhid/article/view/2262
work_keys_str_mv AT julieschanz rarecytogeneticabnormalitiesinmyelodysplasticsyndromes
AT friederikebraulke rarecytogeneticabnormalitiesinmyelodysplasticsyndromes
AT detlefhaase rarecytogeneticabnormalitiesinmyelodysplasticsyndromes