Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ What are myelodysplastic syndromes (MDSs)? ++ Table Graphic Jump LocationFavorite Table | Download (.pdf) | Print Key concept MDSs are a group of heterogeneous myeloid hematopoietic disorders characterized by ineffective hematopoiesis and increased risk of transformation to acute myelogenous leukemia (AML). Most patients with MDS die of causes related to the disease. The median age of patients with MDS is 70–75 years.1 Clinical scenario A 71-year-old man is admitted to a hospital with generalized weakness and dyspnea on exertion. On physical examination, the abdominal reveals no organomegaly. His hemoglobin level is 6 g/dL, and the rest of his blood work is normal. There is no obvious evidence of blood loss. A bone marrow examination shows normocellular marrow with dysplastic changes in the megakaryocytes and erythroid series. Increased promyelocytes with granules (14.6%), eosinophils (5.6%), and basophils (7%) are observed. Karyotype is normal (46,XX). Diagnosis is MDS with eosinophilia and basophilia (refractory cytopenia with multilineage dysplasia). Action items Review whether a patient diagnosed with MDS meets minimal diagnostic criteria2: Stable cytopenia (6 months, or 2 months if there is a specific MDS karyotype or bilineage dysplasia) Exclusion of other disorders as cause of cytopenia and/or dysplasia or both At least 1 of the 3 MDS-related (decisive) criteria: Dysplasia (>10% in ≥1 of 3 major bone marrow lineages) A blast count of 5%–19% Specific karyotype, such as del(5q), del(20)q, +8, or –7del (7q) Co-criteria help to confirm diagnosis: Abnormal bone marrow immune histology, immunohistochemistry Abnormal flow cytometry Abnormal CD34 expression Fibrosis Myeloid clonality Dysplastic megakaryocytes Discussion MDS is usually suspected when there is cytopenia in a routine peripheral blood count. A bone marrow aspiration and biopsy follows, with a manual count of blasts, which is essential for risk assessment. Cytogenetic analysis assists in predicting risk and selecting therapy. Risk is calculated using the International Prognostic Scoring System (IPSS). Patients with low and intermediate-1 (INT-1) scores are treated to improve transfusion needs. Patients with higher scores, intermediate-2 (INT-2) or higher, are treated with interventions patterned after AML therapy. The revised IPSS score (IPSS-R) includes a new cytogenetic risk classification that divides patients into 5 categories. It is used more for prognosis than for deciding therapy, since all the approved treatments for MDS have been approved in clinical trials using the IPSS. Pearl IPSS criteria are used to decide therapy and IPSS-R criteria to evaluate prognosis References Montalban-Bravo G, Garcia-Manero G. Myelodysplastic syndromes: 2018 update on diagnosis, risk-stratification and management. Am J Hematol 2018;93:129-47. Valent P, Horny HP, Bennett JM, et al. Definitions and standards in the diagnosis and treatment of the myelodysplastic syndromes: consensus statements and report from a working conference. Leuk Res 2007;31(6):727-36. ++ What is included in the evaluation of a patient with a myelodysplastic syndrome (MDS)? ++ Table Graphic Jump LocationFavorite Table | Download (.pdf) | Print Key concept A clear picture of the MDS patient’s clinical status is essential for diagnosis, prognosis, and to ... GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. What is MyAccess? Create a FREE MyAccess profile to: Use this site remotely Bookmark your favorite content Track your self-assessment progress and more!