Mutations in genes encoding for the different spliceosome components (SF3B1, serine/arginine-rich splicing factor 2 (SRSF2), U2 small nuclear RNA auxiliary factor1 (U2AF1), and zinc finger CCCH-type, RNA binding motif and serine/arginine rich 2 (ZRSR2)) are recurrent events in MDS and pathophysiologically can lead to an aberrant alternative splicing through activation of incorrect splice sites or intron retention of downstream genes, inducing functional haploinsufficient expression in some instances, similar to chromosomal deletions, epigenetic silencing, or inactivating mutations [85,86,87]. This evidence concerns the gene SRSF2 and myelodysplastic syndrome.