TEMPI syndrome (telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting) is a rare paraneoplastic syndrome associated with MGUS or MM, which may be confused with polycythemia vera due to erythrocytosis caused be extremely elevated erythropoietin levels [71]. This evidence concerns the gene EPO and acquired polycythemia vera.