Sykes et al proposed the term TEMPI Syndrome to describe a systemic disorder in patients with MGUS (monoclonal gammopathy of uncertain significance).
Features:
(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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Specialty: Hematology Oncology
ICD-10: ,
