At the present time, there is a lot of information about clonal plasma cell processes, def ned as the plasma cell dyscrasiаs. We present a case of TEMPI syndrome, which was f rst detected in Russia and belongs to a rare form of plasma cell dyscrasia, with a description of the clinical symptoms, difficulties in differential diagnosis in the patient, and the genetic form of thrombophilia detected in this syndrome. The main manifestations of TEMPI syndrome are telangiectasias, erythrocytosis, monoclonal gammapathy, perinephric f uid collections, and intrapulmonary shunting. Currently, the mechanisms of the systemic effect of monoclonal secretion in this form of plasma cell dyscrasia have not yet been clearly established, and moreover, therapeutic approaches to its treatment have not been developed. The use of bortezomib in the onset of the disease and, further, as part of rotating courses with dexamethasone and cyclophosphane, gave a clinical and hematological effect.