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Myelodysplastic syndrome

Key sources
The following summarized guidelines for the evaluation and management of myelodysplastic syndrome are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2021), the European Society of Medical Oncology (ESMO 2021), and the Blood and Marrow Transplant Clinical Trial Group (BMT CTN/IMDSF/EBMT/ELN 2017).
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Guidelines

1.Screening and diagnosis

Diagnosis
As per BSH 2021 guidelines:
Suspect MDS in patients with otherwise unexplained cytopenias or macrocytosis.
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Recognize that the initial assessment of a patient with unexplained cytopenias may not confirm a diagnosis of MDS. Consider obtaining further follow-up and reassessment to reach a firm diagnosis.
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2.Classification and risk stratification

Classification: classify all cases of MDS according to the WHO classification.
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  • Prognosis

3.Diagnostic investigations

Initial evaluation
Evaluate patients with suspected MDS with a minimum set of investigations and for alternative causes of marrow dysplasia.
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Elicit a detailed clinical and family history to identify potential cases of MDS with germline predisposition.

4.Diagnostic procedures

Biopsy and cytogenetic analysis: as per BSH 2021 guidelines, obtain bone marrow cytogenetic analysis in all patients with suspected MDS undergoing a bone marrow examination.
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5.Medical management

Erythropoiesis-stimulating agents: as per BSH 2021 guidelines, consider offering a trial of ESAs in patients with IPSS low or intermediate-1 (or IPSS-R very low, low, or intermediate with a score up to 3.5) MDS with symptomatic anemia, or asymptomatic anemia and hemoglobin < 100 g/L fulfilling the criteria for a high or intermediate predictive Nordic score for response.
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  • Thrombopoietin receptor agonists

  • Lenalidomide

  • Hypomethylating agents

  • High-intensity chemotherapy

  • Antithymocyte globulin

  • Luspatercept

  • Iron chelation therapy

  • Supportive care

6.Nonpharmacologic interventions

Psychosocial support: assess and address emotional health needs continually. Re-iterate disease-specific information regularly. Offer information regarding how to access MDS patient support groups.

7.Therapeutic procedures

Red blood cell transfusion: administer RBC transfusions to improve symptomatic anemia.
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  • Platelet transfusion

  • Stem cell transplantation (indications)

  • Stem cell transplantation (timing)

  • Stem cell transplantation (donor selection)

  • Stem cell transplantation (pre-transplant management)

  • Stem cell transplantation (post-transplant management)

8.Follow-up and surveillance

Indications for specialist referral
As per BSH 2021 guidelines:
Review cases of MDS with a hematologist with a specialist interest in MDS and refer them for a second opinion if the patient or clinician desires.
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Refer patients with MDS with suspected germline predisposition to a clinical geneticist.

9.Quality improvement

Reporting: report all cases of MDS to the national cancer registry and MDS-specific registries if available.