Pericarditis in Beta-Thalassemia / Hemoglobin E: A Case Report

Abstract

The serious and common cardiac complication in cases with severe beta-thalassemia/hemoglobin E is cardiomyopathy due to the iron overload whereas pericarditis with or without pericardial effusion has been rarely reported. Herein we reported a Thai woman of 45 years of age who was proved to have beta-thalassemia/Hb E disease since childhood, presenting with fatigue and dyspnea without fever for one week. She was examined to have the typical thalassemic facy with anemia, distant heart sound without pericardial rub, huge hepatomegaly, tense ascites and leg edema. The chest film showed cardiomegaly without lung infiltration, the ultrasonography of the abdomen additionally revealed a 6.2 cm homogeneous mass at the left kidney supposed to be the extramedullary hematopoiesis, and the echocardiography suspected hemochromatosis with cardiomyopathy and massive pericardial effusion. Her blood tests included Hb 4.6 g%, WBC 7,340/mm³, ESR 92 mm/hour, ferritin 5,214 ng/mL, AST 80 U/L, ALT 70 U/L, albumin 3.3 g%, normal ASO titer, LDH 1,247 U/L. And anti-dS DNA was not found for two times. The pericardial window yielded the straw-colored fluid of 1,000 ml, WBC 7,800/mm³, N 81 % and its pathological diagnosis was acute and chronic pericarditis. The common causes of pericarditis such as tuberculosis, rheumatic fever, SLE, were tested negative. She was presumably diagnosed as having pericarditis in severe beta-thalassemia/Hb E disease and mild transaminitis from the iron overload. She was treated with the pericardial window and drainage, red blood cell transfusion, diuretics, antibiotics, deferiprone and anti-tuberculous drugs. She could be discharged but re-admitted again within one month because of the recurrent pericardial effusion.  She did not respond to treatments again and passed away on the second day of re-admission. The autopsy was not allowed.