Hematologic Complications of Myelodysplastic Syndromes (MDS)

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By Emcypedia

1. Myelodysplastic Syndromes (MDS)

① Definition and Characteristics

  • Myelodysplastic syndromes (MDS) refer to a group of hematologic malignancies characterized by dysplastic cell maturation and isolated or multiple cytopenias (anemia, neutropenia, thrombocytopenia)
  • MDS shares clinical and pathological features with acute myeloid leukemia (AML), but the proportion of blasts in peripheral blood and bone marrow is lower in MDS (<20% by definition)
  • As a result, patients with MDS are at risk for symptomatic anemia, infections, bleeding, varying degrees of bone marrow failure, and progression to AML
  • The majority of morbidity and mortality in MDS is due to complications from cytopenias rather than transformation to AML

2. Hematologic Complications of Myelodysplastic Syndromes (MDS)

① Evaluation of Anemia

  • More than 75% of MDS patients develop anemia, with half having hemoglobin (Hb) levels below 10 g/dL.
  • Anemia may present with symptoms such as fatigue, anorexia, weight loss, angina, dizziness, dyspnea, headache, and cognitive impairment.
  • Causes of anemia should be evaluated, including blood loss, hemolysis, renal failure, medications, nutritional deficiencies, thyroid dysfunction, autoimmune diseases, and anemia of chronic disease.
  • Diagnostic workup includes:
    • Complete blood count (CBC), peripheral blood smear, reticulocyte count, and reticulocyte production index (RPI) calculation.
    • Evaluation of renal and thyroid function, serum iron, transferrin, ferritin, folate, vitamin B12, copper, and erythropoietin (EPO) levels.

② Treatment of Anemia

For symptomatic patients, treatment is based on MDS risk category, RBC production level (RPI), and serum EPO levels.

  • Low/very low/intermediate risk MDS (IPSS-R ≤4.5 points)
    • If RPI <2 and serum EPO ≤500 mU/mL → Initial treatment with erythropoiesis-stimulating agents (ESAs) is recommended.
    • If RPI ≥2 or serum EPO >500 mU/mL → RBC transfusion or ESA with/without myeloid growth factors may be considered.
  • High/very high-risk MDS (IPSS-R >4.5 points)
    • Chronic transfusion therapy alongside definitive treatment of the underlying disease is suggested.
    • Correction of contributing factors (e.g., bleeding, hemolysis) is necessary.
  • During transfusion, leukoreduced blood products are recommended to reduce the risk of alloimmunization, febrile transfusion reactions, cytomegalovirus (CMV) and other viral infections, and immunosuppression.

③ Infection

  • Infection is a major cause of morbidity and mortality in MDS patients.
  • Increased infection risk is associated with neutropenia and granulocyte dysfunction.
  • Bacterial infections, particularly those involving the skin, are common.
  • Neutropenic patients (absolute neutrophil count <500/microL) require urgent evaluation, blood cultures, and immediate empiric antibiotic therapy.

④ Bleeding

  • Bleeding in MDS may result from thrombocytopenia, qualitative platelet dysfunction, or coagulation disorders.
  • Platelet transfusion recommendations:
    • Prophylactic transfusion for asymptomatic patients with platelet counts <10,000/microL
    • Higher threshold for patients with active bleeding, fever, severe infection, pulmonary dysfunction, coagulopathy, or CNS abnormalities.

Reference: UpToDate.com

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