INTRODUCTION: Myelodysplastic syndromes (MDS) encompass
a heterogeneous group of clonal haematopoietic disorders
characterised by chronic and progressive cytopenias resulting from
ineffective haematopoiesis. Treatment is complicated by differences
in disease mechanisms in different subgroups, variable clinical
phenotypes and risk of progression to acute myeloid leukaemia.
RATIONALE: Changes in disease classification, prognostic scoring
systems, the availability of novel treatment options and the absence
of South African guidelines for the diagnosis and management of
these complex disorders underpinned the need for the development
of these recommendations.
METHODS: These recommendations are based on the opinion of a
number of experts in the field from the laboratory as well as clinical
settings and came from both the private and institutional academic
environments. The most recent literature as well as available
guidelines from other countries were discussed and debated at a
number of different meetings held over a 2-year period.
RESULTS: A comprehensive set of recommendations was developed
focusing on risk stratification, supportive management and specific
treatment. Novel agents and their indications are discussed and
recommendations are made based on best available evidence and
taking into account the availability of treatments in South Africa.
CONCLUSION: Correct diagnosis, risk stratification and
appropriate therapeutic choices are the cornerstones of success in
the management of patients with MDS.
