Scientific programme

PS04  Plenary Session
DIAGNOSIS AND PROGNOSIS OF MDS / NEW CHALLENGES & NEW APPROACHES

04-May-2017 11:30 13:00
 
 
Abstract:
WHO CLASSIFICATION 2016 FOR THE MYELODYSPLASTIC SYNDROMES (MDS): MAIN CHANGES

A refinement of the classification of the MDS has been proposed by the WHO working group in 2016 including a new more precise nomenclature, the reintroduction of the category multilineage dysplasia with ring sideroblasts, the introduction of SF3B1 mutations as a classifier for patients with <15%, but >5% ring sideroblasts, the inclusion of MDS with del(5q) with one additional non chromosome 7 anomaly in to the MDS del(5q) group, and the definition to assess the medullary blast count, namely the blasts should be based on all nucleated cells neglecting the amount of erythroid cells. Besides that, cell counts influencing the categorization have been introduced. In addition, a CMML category with less than 5% medullary blasts (CMML 0) has been introduced and RARS-T has been recognized as a distinct entity. As a result of the new WHO proposals, haematologists, pathologists, human geneticists should be taken into account the following procedures for a proper diagnosis of MDS:

  1. cell counts (number of cytopenias, MDS-U or other MDS type?)
    2. at least 2 full differential counts (monocytosis?, CMML 0, 1, 2 ?, percentage of  peripheral blasts, MDS-U, MDSEB 1,2 ?)
    3. assessment of the number of dysplastic lineages by single cell morphology (>10% dysplastic cells per lineage required, assessment of presence and percentage of ring sideroblasts (including iron staining) (MDS SLD or MDS MLD, MDS RS SLD or MDS RS MLD?)
    4. assessment of the percentage of medullary blasts based on all nucleated cells in the marrow (exact percentage needed for IPSS-R, 0-2, 3-4, 5-9, 10-19%)
    5. analysis of SF3B1 at least in cases with some, but less than 15% ring sideroblasts
    6. karyotype banding of at least 20, better 25 metaphases (MDS del(5q)?, aberration in case of weak signs of dysplasia, MDS-U?)

After producing of these parameters, a proper categorization of the MDS cases according to the WHO 2016 proposals is possible. This new classification allows an even better categorization of MDS patients into more homogenous groups, providing important information with regard to the biology of the disease, prognostic behavior, and therapeutic intervention

 

 
Co-authors
C. Strupp 1, C. Aul 2, U. Germing 3
1Heinrich-Heine University Düsseldorf, Dept. of Hematology- Oncology- and Clinical Immunology, Düsseldorf, Germany
2JOhannes Hospital Duisburg, Hematology- Oncology- and CLinical Immunology, Duisburg, Germany
3Heinrich.Heine University Düsseldorf, Hematology- Oncology- and Clinical Immunology, Düsseldorf, Germany