A 69-year-old man presented to our hospital with shortness of breath and cough. He had a history of essential hypertension but no known significant cardiovascular or pulmonary disease. Almost 1 year before the patient was diagnosed with acute myeloid leukemia with detectable mutations of the nucleophosmin 1 (NPM1) and FLT3 internal tandem duplication (FLT3-ITD) gene. After induction chemotherapy, bone marrow aspiration revealed refractory acute myeloid leukemia with blast persistence, and antileukemic therapy was switched to the multikinase inhibitor sorafenib, resulting in cytologic remission. Subsequently he underwent allogeneic hematopoietic stem cell transplantation (HSCT) and was successfully treated with sorafenib and donor lymphocyte infusions for an early posttransplant leukemic relapse. Before the current presentation, the last leukemia-specific treatment was terminated more than 3 months earlier, and latest laboratory testing of peripheral blood yielded a cytologic remission with a complete donor chimerism.
Assessment
Chest radiographic imaging showed a pulmonary infiltrate and newly developed cardiomegaly. After admission for intravenous antibiotic treatment of pneumonia, the patient acutely developed tachycardia. Electrocardiography demonstrated atrial flutter, and the clinical condition deteriorated with progressive signs and symptoms of acute heart failure, with a functional capacity New York Heart Association class IV.
Transthoracic echocardiography revealed a moderate circumferential pericardial effusion without hemodynamic compromise and 3 large pericardial solid masses located next to the right ventricle, right atrium, and surrounding the ascending aorta (Figure 1).
Cardiac magnetic resonance imaging confirmed the moderate circumferential pericardial effusion and showed the presence of a large epicardial mass (79 × 30 mm) localized at the right ventricle, with infiltration of the ventricular wall. Another mass was localized at the coronary sulcus, with contact to the left coronary artery. These masses were inhomogeneous, slightly hyperintense on T1-weighted images, showed a heterogeneous hypointense signal on T2-weighted images, and were isointense in the native steady-state free precession-sequences. Functional analysis revealed a normal left ventricular ejection fraction, whereas the right ventricular systolic function was severely reduced, exhibiting hypokinesia of the involved right ventricle parts. The mass enhanced less than the adjacent myocardium.
Additionally, a contrast-enhanced volume perfusion computed tomography scan was performed, disclosing multiple further masses with additional involvement of the left ventricular myocardium and showing marked differences with regard to perfusion of the affected tissue (Figure 2).
On the basis of the patient’s history with induction failure and relapse after allogeneic HSCT, recurrence of acute myeloid leukemia with an isolated cardiac manifestation was considered a likely diagnosis. Therefore, a myocardial right ventricular septum biopsy was obtained, but histopathologic examination did not reveal any signs of malignancy or infection. Unfortunately, the patient died shortly after, secondary to perforated sigmoid diverticulitis and consecutive multiorgan failure.
To read this article in its entirety please visit our website.
-Daniela Dörfel, MD, Maik Häntschel, MD, Birgit Federmann, MD, Susanne Haen, MD, Falko Fend, MD, Iris I. Müller, MD, Helmut R. Salih, MD, Wichard Vogel, MD, Lothar Kanz, MD, Marius Horger, MD
This article originally appeared in the August 2016 issue of The American Journal of Medicine.
