Myelodysplastic Syndrome in a Patient With Cronkhite‐Canada Syndrome

Cronkhite-Canada Syndrome (CCS) is a rare non-hereditary gastroenterological polyposis syndrome without a clear etiology. Myelodysplastic syndromes (MDS) are clonal hematological disorders. Immune dysregulation has been posited as a contributor to the development of both. A 70-year-old man developed chronic diarrhea, dysgeusia, and onycholysis. Endoscopic evaluation and testing to exclude hereditary polyposis syndromes resulted in a diagnosis of CCS. Approximately 1 year later, he was noted to have persistent anemia, which was ultimately diagnosed as MDS. He was evaluated with a myeloid disorder next-generation sequencing panel and found to have mutations in <i>IDH2</i>, <i>SRSF2</i>, and <i>JAK2</i>. This is the second reported case characterizing concomitant MDS and CCS. This is the first case to report the development of MDS after the diagnosis and treatment of CCS. A successful management strategy for both disorders is outlined within this case report.