Sykes et al proposed the term TEMPI Syndrome to describe a systemic disorder in patients with MGUS (monoclonal gammopathy of uncertain significance).



(T) telangiectasias, prominent over the face, trunk and upper extremities

(E) erythrocytosis with elevated serum erythropoietin concentration

(M) monoclonal gammopathy, often IgG kappa, with less than 10% plasma cells in the bone marrow

(P) perinephric serous fluid, between the kidney and the renal capsule

(I) intrapulmonary shunt with hypoxemia


A patient responded to therapy with bortezomib, a proteosome inhibitor.


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