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KEY CONCEPTS

KEY CONCEPTS

  • imageMyelodysplastic syndromes (MDS) primarily affect elderly adults.

  • imageThe exact cause of MDS is unknown and is probably multifactorial. MDS have been associated with host-specific characteristics, environmental, lifestyle, and therapeutic exposures.

  • imageGenomic instability drives MDS development and progression. The clonal population of cells manifested as MDS results from enhanced self-renewal of a hematopoietic stem cell or acquisition of self-renewal in a progenitor cell, increased proliferative capacity in the abnormal clone, impaired cell differentiation, evasion of immune regulation, and antiapoptotic mechanisms in the disease-sustaining cell.

  • imageMost patients with MDS present with fatigue, infection, bleeding/bruising, lethargy, or other symptoms related to cytopenias.

  • imageThe prognosis of patients with MDS is variable and depends on the biology of the MDS and host characteristics. Overall survival time ranges from a few months to several years and is most accurately estimated with the International Prognostic Scoring System—Revised (IPSS-R).

  • imageThe goals of therapy for MDS are to change the natural history of the disease, reduce the number of red blood cell transfusions, and improve quality of life.

  • imageLenalidomide should be considered for patients with MDS that harbors a del(5q) clone and is particularly beneficial in those with symptomatic anemia.

  • imagePatients with lower-risk MDS and symptomatic anemia who have a serum erythropoietin level ≤500 mU/mL (U/L) are suitable candidates for an erythropoietin stimulating agent with or without growth factor support.

  • imageA subset of lower-risk MDS patients respond well to antithymocyte globulin (ie, immunosuppressive therapy), which is most effective in patients who have a hypocellular marrow, MDS that expresses HLA DR15 with trisomy 8 as the sole cytogenetic abnormality, refractory anemia, and are younger than 60 years old.

  • imageFurther evaluation is required to determine optimal hypomethylating agent treatment regimens.

  • imageAllogeneic hematopoietic stem cell transplantation offers potentially curative therapy to patients with MDS who have a donor and are healthy enough for the procedure.

PATIENT CARE PROCESS

Patient Care Process for Myelodysplastic Syndromes

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Collect

  • Patient chief complaint (eg, fatigue, easy bruising)

  • Patient characteristics (eg, age, sex, pregnant, weight)

  • Patient medical history (personal including blood transfusion history, sexual history, reproductive history including whether the patient is still menstruating and has her uterus (if female), and family history minimum of 3 generations)

  • Social history (eg, tobacco/ethanol use), employment status and history

  • Current medications including over-the-counter aspirin/nonsteroidal anti-inflammatory drug use, herbal products, dietary supplements, and prior chemotherapy, radiotherapy, or granulocyte colony-stimulating factor use

  • Full review of systems

  • Objective data

    • Blood pressure (BP), heart rate (HR), respiratory rate (RR), height, weight

    • Labs including complete blood count with differential, peripheral blood smear, reticulocyte count, serum vitamin B12, RBC folate and copper, HIV, TSH, ferritin, iron, TIBC, LDH

    • Bone marrow biopsy procedure: morphologic evaluation, karyotyping, consider molecular genetic evaluation for TP53, ASXL1, etc.

    • Serum erythropoietin level (for lower risk MDS)

Assess

  • Karyotype and cytogenetics as well as any identified somatic mutation

  • Degree of cytopenias and percentage of blasts

  • Determine the WHO subtype of MDS according ...

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