BLOC1S3 and Li-Fraumeni syndrome: While the cause and effect of germline RP haploinsufficiency and somatic Tp53 LOF mutations in DBA-associated cancer is yet to be determined, the concordance of cancer types in DBA (faulty ribosome biosynthesis) and the germline Tp53 LOF-mutated Li-Fraumeni syndrome (LFS) as opposed to cancers observed in other IBMFS [3], Fanconi anemia (FA; faulty DNA repair) and dyskeratosis congenita (DC; faulty telomere maintenance), suggests that p53 LOF may contribute to cancer predisposition in DBA.