The National Comprehensive Cancer Network (NCCN) has published updates to their clinical practice guidelines for myelodysplastic syndromes (MDS), including updates to initial evaluation, additional testing, and prognostic categories. 

For the section on initial evaluation (MDS-1), the bullet stating “consider molecular testing for recurrently mutated MDS genes in appropriate clinical contexts” has been removed.  Two bullets were added: “Consider genetic testing for somatic mutations (ie, required mutations) in genes associated with MDS,” and “Consider additional molecular and genetic testing for hereditary hematologic malignancy predisposition in a subset of patients, particularly in younger patients.”

The statement “Consider congenital bone marrow syndromes (eg, dyskeratosis congenita, Shwachman-Diamond syndrome),” was removed from footnote F and “CSA may appear late due to lyonization in X-linked sideroblastic anemia (not limited to younger patients),” was added.

For additional testing (MDS-2), a new bullet was added: "CMV-safe (CMV-negative or leukopheresed) blood products are recommended whenever possible for CMV-negative transplant candidates." 

For prognostic category – very low, low, and intermediate (MDS-4), the pathway to follow was clarified: (serum EPO > 500 mU/mL and poor probability to respond to immunosuppressive therapy [IST]) if progression on all recommendations in the serum EPO ≤500 mU/mL branch and the serum EPO >500 mU/mL and a good probability to respond to IST branch.

For evaluation of related anemia and treatment of symptomatic anemia (MDS-5), the treatment recommendations were revised for patients with a serum EPO ≤500 mU/mL and ring sideroblasts <15% who had no response to an erythropoiesis-stimulating agent (ESA) alone. Revised recommendations now suggest adding lenalidomide +/- G-CSF (which is consistent with recommendations on MDS-4).

For prognostic category – intermediate, high, very high (MDS-6), “Donor stem cell source available” was removed from the pathway.

For supportive care (MDS-7), under cytokines, a sub bullet was added stating "EPO refers to the following agents: epoetin alfa and epoetin alfa-epbx." "G-CSF or GMCSF" was changed to "G-CSF" and a sub bullet was added that states "G-CSF refers to the following agents: filgrastim, filgrastim-sndz, and tbo-filgrastim."

Under the section for the 2016 WHO Classification of MDS (MDS-C, page 1 of 2), MDS with multilineage dysplasia, bone marrow, unilineage erythroid dysplasia, isolated del(5q), < 5% blasts was revised to read, "<1 5% ring sideroblasts (or < 5% ring sideroblasts if SF3B1 mutation present)."MDS with isolated del(5q)was modified and "± one other abnormality except -7/del(7q),” was added. “Provisional WHO category” was added for refractory cytopenia of childhood. 

For Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN) WHO Classification (MDS-A, page 2 of 2), the table was updated and appropriate references are provided. 

For the section on genes frequently somatically mutated in MDS (MDS-C, page 1 of 7), the table was updated. For genes associated with hereditary myeloid malignancies (MDS-C), the information and tables are new to the guidelines on pages 3-6.—Janelle Bradley