Effect of vasodilators in patient with pulmonary hypertension associated with hemolytic anemia

Pulmonary arterial hypertension (PAH) has been described to associate with hemolytic anemia in updated clinical classification of pulmonary hypertension. A 56-year-old woman, diagnosed with warm antibody autoimmune hemolytic anemia (AIHA), was treated with oral corticosteroids at the Department of Hematology, Osaka University Hospital for 30 years. Her AIHA worsened 3 months before the admission, and she was treated with rituximab and cyclosporine in combination with prednisolone. Soon after she left the hospital, she developed dyspnea on effort and leg edema, therefore she was re-admitted to the Department of Cardiovascular Medicine. Echocardiogram and cardiac catheterization demonstrated PAH associated with AIHA. She was treated with three types of vasodilatory agents, resulting in an improvement in pulmonary arterial pressure and pulmonary vascular resistance after 6 weeks. A few weeks after she left the hospital, her hemolytic anemia became in remission without intensifying AIHA therapy, and did not worsen for a year of follow-up. Although corticosteroids are the first-line treatment for AIHA, medications for PAH should be considered when the first-line therapy for AIHA failed to improve PAH.