Genetic testing, use of erythropoiesis-stimulating agents, the latest advice on transplantation and more detailed recommendations on use of iron chelators have all been updated in new UK guidelines on adult myelodysplastic syndromes.
The recommendations from the British Society for Haematology– now split into two documents one on diagnosis and one on management – point out that molecular testing using targeted mutation panels is now widely available, increasingly affordable and should be considered in all patients.
While diagnosis in these patients can be challenging, new technologies such as genomic testing may help provide additional guidance, although should not be used in isolation of other diagnostic tests, the recommendations state.
And mutational analysis is recommended where it might help clarify sub-classiﬁcation of disease, identify prognostic mutations in the relevant setting or guide management decisions.
Results from recent randomised studies on erythropoiesis-stimulating agents (ESA) mean a trial of the treatment should be considered in very low, low or intermediate grade MDS with symptomatic anaemia or asymptomatic anaemia who meet the criteria.
The BSH guidelines which were last updated in 2014 state that for maximum benefit, ESA treatment should be started as soon as appropriate after diagnosis of MDS but before established transfusion dependence.
An eight-week period of a starting dose, with eight weeks of higher doses if needed and a final eight weeks with the addition of granulocyte-colony stimulating factor in the case of no response should be done before the patient is considered to have failed treatment, the recommendations said.
Iron chelation has been a “hotly debated topic” over the years, said guideline author Dr Sally Killick, Consultant Haematologist at University Hospitals Dorset.
“Expert opinion is that deferasirox, although only licensed second line in MDS is drug of choice based on tolerability, compliance and mature safety data,” she added.
Although the guideline committee cover the latest evidence on luspatercept as a potential next stage, they note that has not yet been approved for use in many countries.
An accompanying editorial points out that updated WHO definitions of MDS with Isolated del(5q) have been included in the new recommendations with first-line treatment for symptomatic anaemia in these patients still ESA.
Lenalidomide should be reserved for isolated del(5q) MDS patients who have failed or are unresponsive to ESA.
There are also more “precise” recommendations on use of transplant in lower risk MDS as well as around overall patient selection and donor choice, in this “timely update” the editorial notes.