Severe mechanical hemolysis in a patient with thalassemia minor who had undergone inappropriate splenectomy

Excerpt

An 82-year-old Italian-American man with a history of β-thalassemia minor underwent coronary artery bypass grafting and bioprosthetic mitral valve replacement in 2003. In January 2016, he was admitted to an outside facility after a syncopal episode. Over the previous 12 months, he had developed increasing fatigue and dyspnea. Reportedly, serial echocardiograms had been unremarkable. Moreover, in early 2015, he had been found to have worsening hemolytic anemia. Direct antiglobulin test (DAT) was negative and a search for underlying autoimmune disease or malignancy unrevealing. Ultimately, the patient underwent bone marrow aspiration and biopsy, the result of which was unremarkable. He was initially treated with periodic red blood cell (RBC) transfusions and corticosteroids. Subsequently, despite a negative DAT, unremarkable bone marrow findings, and the lack of response to corticosteroids, splenectomy was pursued, again without improvement in hemoglobin levels. …