Monoclonal Gammopathy with Known Significance in TEMPI Syndrome: Case Report and Minireview of the Literature

TEMPI (telangiectasias, elevated erythropoietin level, and erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting) syndrome is a rare plasma cell neoplasm with associated paraneoplastic features. We diagnosed a new case with IgG kappa paraproteinaemia and a bone marrow plasma cell ratio not reaching the criteria of smoldering myeloma, but harboring translocation t(11;14). Unfortunately, the patient succumbed to central nervous system thrombosis and bleeding, therefore treatment results could not be reported. This sequence of events prompted a reevaluation of the currently available knowledge of TEMPI syndrome. Annals of Case Reports Harasztdombi J, et al. Ann Case Rep: 7: 779. www.doi.org/10.29011/2574-7754.100779 www.gavinpublishers.com