Myelodysplastic Syndrome - myelodysplastic syndrome treatment, mds, prognosis, symptom

These are a heterogeneous group of myeloid disorders characterized by abnormal morphology and maturation in the bone marrow. They are much more common in older subjects, and often occur in response to bone marrow damage or acquired genetic rearrangement. MDS is as common as acute myeloid leukaemia and is being recognized with increasing frequency. The natural history of MDS is variable, but there is a high morbidity and mortality owing to bone marrow failure, and transformation into acute myeloblastic leukaemia occurs in about 30% of cases.

There are a number of different types of MDS:

• refractory anaemia (RA)
• refractory anaemia with ringed sideroblasts (> 15% ringed sideroblasts - RARS)
• refractory anaemia with an excess of blasts (blasts 5-20% - RAEB)
• refractory anaemia with an excess of blasts in transformation (blasts 20-30%) (RAEB-T)
• chronic myelomonocytic leukaemia (CMML).

Diagnosis and clinical features of myelodysplastic syndromes disease

MDS occurs mainly in the elderly and presents with symptoms of anaemia, infection or bleeding due to pancytopenia. Serial blood counts show evidence of increasing bone marrow failure with anaemia, neutropenia, monocytosis and thrombocytopenia, either alone or in combination. In CMML, monocytes are > 1 × 10 9 /L and the WBC count may be > 100 × 10 9 /L.

The bone marrow usually shows increased cellularity despite the pancytopenia. Dyserythropoiesis is present, and granulocyte precursors and megakaryocytes also have abnormal morphology. Ring sideroblasts are present in all types. In RAEB and RAEB-T, the number of blasts in the bone marrow is increased, and the prognosis is worse than in those types with a normal number of blast cells (< 5%).

Management of myelodysplastic syndromes

Patients with < 5% blasts in the bone marrow are usually managed conservatively with red cell and platelet transfusions and antibiotics for infections, as they are needed. Haemopoietic growth factors (e.g. erythropoietin, G-CSF) may be useful in some patients.

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