The 17p-Syndrome: A Distinct Myelodysplastic Syndrome Entity?

Abstract

The 17p- syndrome is a subset of myelodysplastic syndrome characterized by "typical" dysgranulopoïesis, combining a pseudo-Pelger-Hüet and a deletion of the short arm of chromosome 17. We describe two patients; one with de novo myelodysplastic syndrome (RAEB), one with secondary MDS (RAEB-T). Both showed a 17p- deletion resulting from tanslocations involving 17p associated with an additional complex cytogenetics, and both of them had a particular type of dysgranulopoiesis, combining pseudo-Pelger-Hüet anomaly.