Myelodysplastic syndrome

Heterogeneous group of clonal stem cell disorders, characterised by ineffective haematopoiesis.

Primarily a disease of older adults. Can present with symptoms of anaemia, leukopenia, and thrombocytopenia, but often found during routine laboratory investigations in asymptomatic patients.

Diagnosed when bone marrow demonstrates significant dysplasia, clonal cytogenetic abnormality, quantitative changes in at least one of the blood cell lines, and blasts <20%.

Treatment often based on supportive measures, including red cell and platelet transfusions. Chemotherapy agents such as azacitidine, lenalidomide, and decitabine are used for certain patients. Stem cell transplant is the only potentially curative therapy.

May have a prolonged course with anaemia and neutropenic infections, or may progress rapidly to acute myelogenous leukaemia (AML). However, most patients die of infection while in the myelodysplastic syndrome (MDS) stage of their disease.

Myelodysplastic syndrome (MDS) is a group of clonal stem cell disorders, characterised by ineffective and dysplastic haematopoiesis resulting in 1 or more cytopenias, and a varying predilection to develop acute myeloid leukaemia (AML).[1]Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016 May 19;127(20):2391-405. http://www.bloodjournal.org/content/127/20/2391.long?sso-checked=true http://www.ncbi.nlm.nih.gov/pubmed/27069254?tool=bestpractice.com MDS is diagnosed when bone marrow demonstrates significant dysplasia, clonal cytogenetic abnormality, quantitative changes in at least one of the blood cell lines, and blasts <20%. Patients with blasts ≥20% are considered to have AML. MDS can arise primarily without any inciting event or may be related to previous treatment with either chemotherapy or radiation.