Anemia is a syndrome that can occur with bleeding and infections, and can often be seen in middle-aged people. The disease is more common in men than women. MDS has also been described in children. Unfortunately, the incidence of the syndrome is increasing as a result of prolonged human life, radiotherapy, chemotherapy, and increased contact with chemicals. The syndrome includes a group of diseases ranging from refractory (resistance) anemia (RA) to acute leukemia (AML). These diseases, which have been defined separately as refractory anemia, ring sideroblastic anemia, preleukemia, chronic myelomonocytic leukemia (CMML) since the beginning of the twentieth century, have been grouped under the heading of myelodysplastic syndrome (MDS) with the classifications made since the early eighties (FAB – WHO).
In this group of diseases, there are resistant anemias (refractory – RA), which are only associated with low red blood cells, as well as acute and chronic leukemias. Therefore, patients with myelodysplastic syndrome; They can lead very different lives depending on the subgroups of the disease, morphological – cytogenetic anomalies, molecular mutations, age of the patients and comorbid diseases.
In the diagnosis and differential diagnosis of the disease; Cytopenia, bicytopenia or pancytopenia in peripheral blood (decrease in one, two or three groups of blood cells), bone marrow findings often containing myelodysplastic changes, rich in cells (hypercellular), less frequently in cells poor (hypocellular), spleen size in some subgroups Presence (splenomegaly), mutations, cytogenetic abnormalities help.
Differential Diagnosis:
Bone marrow deficiencies ( aplastic anemias , PNH… )
Leukemias ( CML, HCL…)
Anemias ( B12, folic acid deficiencies…)
Leukopenia and thrombocytopenias (ITP, idiopathic cytopenias…)
Others (idiopathic dysplasias, Felty’s syndrome, folic acid antagonists …)
Treatment:
To vary significantly in patients, depending on the characteristics of the subgroups of MDS; We can talk about (specific) and supportive treatments for the disease.
Specific (directly directed at the disease) treatments:
Use of drugs such as cytosine arabinoside, anthracyclines, azacitidine, decitabine, topotecan, isotretinoin, lenalidomide, arsenic trioxide…
Allogeneic stem cell transplantation should also be considered as an important treatment option in patients with MDS. Factors such as the patient’s age, suitable donors (relative – unrelated), subgroups of the disease, and co-morbidities significantly affect the application and success of this treatment option.
Supportive therapies:
Erythrocyte (red blood cell) and platelet (platelet) transfusions, growth factors (G-CSF, GM-CSF), erythropoietin (EPO), iron-binding agents depending on the subtypes of the disease patients can be applied.
Isolation of patients, oral care, use of different antibiotics, antifungal and antiviral drugs against infections are other approaches in treatment.
Prof.Dr.Vasıf Akın UYSAL
Hematology
