SOHO State of the Art and Next Questions: Management of Myelodysplastic Syndromes With Deletion 5q

Abstract

Myelodysplastic syndrome (MDS) with deletion 5q [del(5q)] is a distinct clinical and pathologic disease subset that is exquisitely sensitive to lenalidomide for the treatment of red blood cell transfusion-dependent anemia. Lenalidomide resistance, including primary resistance, occurs by clonal evolution, which is frequently attributable to the presence of somatic mutations in the DNA-binding domain of the TP53 gene. The treatment options after development of resistance to lenalidomide are limited and consist of hypomethylating agents, clinical trials, and allogeneic hematopoietic stem cell transplantation. We discuss evidence-based strategies to devise a treatment algorithm for patients with MDS with del(5q).

Keywords: Del(5q); Hypomethylating agent; Lenalidomide; MDS; Treatment algorithm.