The First Case of TEMPI Syndrome in Japan
Tatsunori Shizuku, Keiji Matsui, Shinya Yagi, Shogo Iwabuchi
Abstract
TEMPI syndrome, a disease entity comprising telangiectasia, erythrocytosis with high erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting, was first described by Sykes et al. in 2011. To our knowledge, only 15 cases have been reported worldwide, none of which were in Japan. We herein report a 47-year-old man who had intractable ascites for 2 and a half years and was referred to our department for a peritoneovenous shunt. In addition to ascites, he had telangiectasia, high erythropoietin, monoclonal gammopathy, and perinephric fluid collection. Thus, this is the first case of TEMPI syndrome in Japan.
Topics & Concepts
MedicineAscitesMonoclonal gammopathyErythropoietinSurgeryInternal medicineMonoclonalMonoclonal antibodyImmunologyAntibodyVascular Malformations and HemangiomasVascular Anomalies and TreatmentsVascular Tumors and Angiosarcomas