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Mutation carriers in 4q-related myelodysplastic syndromes.
Mutations in the TP53 gene are associated with the development of myelodysplastic syndromes (MDS), which are clonal haematopoietic stem cell disorders with heterogeneous clinical manifestations and a median survival of 3 years. C-isochromosome 4q (i(4q)), a reciprocal translocation t(4;14)(q28;q32.3) seen in a subset of MDS, has been shown to be associated with p53 mutations. We therefore set up a laboratory-based study to determine whether i(4q) was associated with TP53 mutations in MDS. A cohort of 62 i(4q) and 10 i(4q)-related complex karyotype (i(4q)-CK) MDS patients (diagnosed in 2002-2003) from the Mayo Clinic was tested for p53 mutations by denaturing high-performance liquid chromatography (dHPLC) and sequencing. In addition, dHPLC was used to investigate a second cohort of 11 i(4q) and eight i(4q)-CK MDS patients (diagnosed in 2006-2007) to determine whether p53 mutations occurred in i(4q)-CK MDS. We identified 11 i(4q) MDS patients with TP53 mutations. In the second cohort of 12 i(4q)-CK patients, we found two additional TP53
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