Rare acquired disorders to consider include the monoclonal gammopathy driven TEMPI syndrome which is characterized by telangiectasias, elevated serum EPO (which might be >5000 mIU/ml) and erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting [87, 88]; erythrocytosis and telangiectasis are often the initial manifestation, therefore obtaining monoclonal protein studies might provide diagnostic clues to this entity. This evidence concerns the gene EPO and telangiectasis.