Constrictive Pericarditis Due to Graft-vs-Host Disease

Abstract

A 29-year-old man experienced a diffuse lichenoid rash that extended to his palms (panel A) and soles, peripheral blood eosinophilia, blurry vision, and recalcitrant generalized edema 2 weeks after donor lymphocyte infusion (DLI) for relapsed T-cell acute lymphoblastic leukemia. Skin biopsy revealed vacuolar interface inflammation consistent with graft-vs-host disease (GVHD). The rash improved with topical corticosteroids; however, the edema gradually worsened during the ensuing weeks, and the patient gained 23 kg. A thorough endocrine/metabolic evaluation for causes of anasarca was unremarkable. Transthoracic echocardiography, which had been completely normal 2 weeks before DLI, revealed a thickened pericardium, paroxysmal ventricular septal motion (supplemental video online linked to this article), high medial mitral annular velocity (panel B), and phasic respiratory changes of mitral valve inflow (E wave velocity; panel C). Cardiac magnetic resonance imaging demonstrated abnormal concentric delayed pericardial enhancement (arrow, panel D). The patient's condition was unresponsive to oral colchicine of 1 month's duration. Because his symptoms attributable to constrictive pericarditis occurred in the setting of GVHD development in other organs after DLI, the patient was treated with a moderate dose of corticosteroids (prednisone, 0.5 mg/kg/d). The anasarca rapidly improved. Six weeks after initiation of prednisone and 6 months after DLI, the patient remained in remission from T-cell acute lymphoblastic leukemia. His symptoms due to chronic GVHD were manageable; however, he died of infectious complications of profound immunosuppression. Pericarditis is an uncommon manifestation of chronic GVHD,1 and, to our knowledge, has been reported after DLI only once previously.2