Pb1976 microangiopathic Hemolytic Anemia Due to Heart Valve Prosthesis

Abstract

Background:Microangiopathic hemolytic anemias (MAHA) are characterized by the presence of hemolytic anemia (HA), related to red blood cell (RBC's) fragmentation, thrombocytopenia and hemosiderinuria due the intravascular hemolysis. The term thrombotic microangiopathy (TMA) is also used to describe syndromes characterized by AHMA, thrombocytopenia, and thrombotic lesions in small blood vessels such as Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic uremic syndrome (HUS). Although TTP and HUS are the prototypes of TMA / MAHA, there are other causes that should not be forgotten, such as MAHA by valvular leak.Aims:Perform the casuistry of the Hematology Service of a Central Hospital for a rare cause of hemolytic anemia.Methods:Parameterized search for the data of patients with MAHA and hemosiderinuria evaluated at a Hematology out patients clinic at a Central Hospital between January 2007 and March 2018.Results:9 patients with MAHA were identified with valvular leak, the mean age at diagnosis was 68 years and 6 were men (66%). All patients had mechanical heart valves: 66% mitral valve, 22% aortic valve and 11% both valves. The time from surgery to diagnosis of AHMA was ∼ 2 years. The mean Hb value was 8.4 mg/dL. All patients had RBC fragmentation. The mean value of DLH was 3773U/L, total bilirubin (TBil) was 1.9 mg/dL. 88% patients were supplemented with oral iron. Only 3 patients increase ∼ 1.5 g of Hb after 3 months of therapy. 1/3 patients were supplemented with EV iron; 1/3 needed blood transfusion. 66% underwent valvular leakage correction and of these 50% significantly improved Hb and hemolysis.Summary/Conclusion:A careful clinical history and the RBC morphology play a central role in the diagnosis of anemia. The MAHA due to leak valvular is a rare entity, but with a tendency to increase. Despite the small number of patients, the authors consider pertinent to present these data because this is a potentially reversible cause of anemia. The disproportionate high DHL level in relation to the TBilis a possible clue to the differential diagnosis of HA.