{"id":5341,"date":"2021-05-15T23:53:42","date_gmt":"2021-05-15T21:53:42","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/hematological-oncology\/myelodysplastic-syndrome-mds\/"},"modified":"2022-02-02T01:19:45","modified_gmt":"2022-02-01T23:19:45","slug":"myelodysplastic-syndrome-mds","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/myelodysplastic-syndrome-mds\/","title":{"rendered":"Myelodysplastic syndrome (MDS)"},"content":{"rendered":"\n

Introduction<\/h4>\n<\/span>\n\n\n

Myelodysplastic syndrome is a group of clonal disorders characterized by ineffective and dysplastic hematopoiesis.<\/p>\n\n\n\n

  • Slow progression.<\/li>
  • Mostly appears in older adults (>70).<\/li>
  • The bone marrow hypercellular, but there is pancytopenia.<\/li>
  • Tends to progress to AML.<\/li>
  • Most cases are primary, but can also appear secondary to chemotherapy or ionizing radiation.<\/li>
  • Characterized by distinct mutations in the hematopoietic stem cells, most frequently in genes involved in DNA methylation (TET2, DNMT3A, IDH1\/2) and RNA splicing (ASXL1, EZH2).
    • The DNA methylation mutations appear in other malignancies, but the RNA splicing is rare outside of MDS.<\/li>
    • Transformation of MDS to AML is typical in ASXL1 mutations.<\/li><\/ul><\/li><\/ul>\n\n\n<\/span>\n

      Classification<\/h4>\n<\/span>\n\n\n

      According to the WHO classification system of 2016, there are 6 major types, based on the following factors:<\/p>\n\n\n\n

      1. % of dysplastic hematopoietic cells in the bone marrow<\/li>
      2. Number of affected lineages (cytopenia)<\/li>
      3. The portion of ring sideroblasts (cells with iron rings around their nucleus) out of the early RBCs<\/li>
      4. The portion of blast cells in the bone marrow<\/li>
      5. Specific chromosomal abnormalities (5q deletion)<\/li><\/ol>\n\n\n<\/span>\n

        Clinical presentation<\/h4>\n<\/span>\n\n\n
        • Early on, asymptomatic without splenomegaly<\/li>
        • Pancytopenia with impaired function of the cells (anemia, recurrent infections, and hemorrhage our of proportion of the cytopenia)<\/li><\/ul>\n\n\n<\/span>\n

          Diagnosis<\/h4>\n<\/span>\n\n\n
          • Bloodwork (demonstrating pancytopenia)<\/li>
          • Reticulocyte count (decreased)<\/li>
          • Blood smear (macrocytic cells, granulocytes with lack of granulation, Pelger-Huet anomaly – bilobed nucleus in leukocytes, dacrocytes <\/strong>– tear-drop RBCs)<\/li>
          • Bone marrow examination (hypercellularity, ring sideroblasts in some cases – required Prussian blue staining, defective granulocytes; in 20% of cases it is hypocellular resembling aplastic anemia)<\/li>
          • Genetic analysis (karyotyping and FISH to detect 5q deletion or loss of chromosome 5\/7 and trisomy 8)<\/li><\/ul>\n\n\n<\/span>\n

            Scoring system<\/h4>\n<\/span>\n\n\n

            IPSS-R is a prognostic scoring system dividing the patients into low-risk, intermediate-1, intermediate-2, and high-risk, based on the bone marrow blast count, karyotyping, and the number of cytopenias.<\/p>\n\n\n<\/span>\n

            Treatment<\/h4>\n<\/span>\n\n\n

            The IPSS-R scoring system can be used for determining the treatment.<\/p>\n\n\n\n

            • Low-risk:
              • Cyclosporin or anti-thymocyte globulin<\/li>
              • Lenalidomide in 5q deletion<\/li>
              • Stem cell transplantation<\/li><\/ul><\/li>
              • High-risk:
                • Stem cell transplantation<\/li>
                • Hypomethylating agents (azacytidine and decitabine, inhibit the methylation of newly formed DNA)<\/li><\/ul><\/li><\/ul>\n\n\n\n

                  For the treatment of the cytopenias:<\/p>\n\n\n\n

                  • EPO and transfusions + iron chelation<\/li>
                  • TPO (eltrombopag)<\/li>
                  • G-CSF<\/li>
                  • Antibiotics<\/li><\/ul>\n<\/span>

                    Introduction<\/h4>

                    Classification<\/h4>

                    Clinical presentation<\/h4>

                    Diagnosis<\/h4>

                    Scoring system<\/h4>

                    Treatment<\/h4><\/div>","protected":false},"excerpt":{"rendered":"

                    Introduction Myelodysplastic syndrome is a group of clonal disorders characterized by ineffective and dysplastic hematopoiesis. Slow progression. Mostly appears in older adults (>70). The bone marrow hypercellular, but there is pancytopenia. Tends to progress to AML. Most cases are primary, but can also appear secondary to chemotherapy or ionizing radiation. Characterized by distinct mutations in the […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5331,"menu_order":3,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5341","page","type-page","status-publish","hentry"],"yoast_head":"\nMyelodysplastic syndrome (MDS) – Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/myelodysplastic-syndrome-mds\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" 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