Treatment with Decitabine Followed By Prednisone for Myelodysplatic Syndrome Concomitant with Sjogren's Syndrome

Myelodysplastic Syndrome (MDS) is rarely concomitant with Sjögren's syndrome (SS) with only 9 previously reported cases in the literature. A case of a previously healthy 31-year-old Chinese female presenting with dysplasia affecting all three lineages without blasts excess showed by bone marrow examination is reported. Autoimmune workup revealed positive antinuclear antibodies and Anti-SSA. Lip biopsy revealed findings consistent with SS. A diagnosis of SS associated with MDS of the refractory cytopenia with multilineage dysplasia (RCMD) subtype was established, and decitabine for five days at 20mg/m² per day was initiated with only slight increase in platelet counts. Four weeks later, another cycle of decitabine for five days at 20mg/m² per day was performed. Unfortunately, infection occurred, and the patient suffered from thrombocytopenia and leucopenia. Then the patient received the treatment of Biapenem and Vancomycin Hydrochloride along with granulocyte colony stimulating factor followed by platelet infusion for two weeks. Significant clinical and laboratory response was achieved. In hopes of improving both response rates and durability of response, the third cycle of decitabine for five days at 20mg/m² per day was performed with again an excellent clinical and laboratory response. However the patient refused further cycle of decitabine due to cost consideration, then prednisone at 10mg per day was used as a basic treatment for SS. It was an amazing feat that the patient was in a state of remission for two years. MDS with SS tends to be incurable. Decitabine alone with prednisone may serve as an effective option to those patients.