Case Reports: Monday, October 24, 2011 |

Restrictive Cardiomyopathy Due to Endocardial Invasion by Advanced Peripheral T-Cell Lymphoma FREE TO VIEW

Tarik Ngab, MD; Nidhi Nikhanj, MD; Mariam Thomas, MD; Robin Wachsner, MD; Nader Kamangar, MD
Chest. 2011;140(4_MeetingAbstracts):60A. doi:10.1378/chest.1118864
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INTRODUCTION: Cardiac involvement secondary to malignant lymphoma has rarely been described as a presenting feature of this disease. We describe an unusual case of a patient with advanced T-cell lymphoma presenting with dyspnea due to a restrictive cardiomyopathy resulting from endocardial tumor infiltration.

CASE PRESENTATION: A 29-year-old Hispanic female with no medical history presented with a 3-month history of fevers, chills, night sweats, and weight loss. Physical exam revealed generalized lymphadenopathy and splenomegaly. The patient underwent an excisional axillary lymph node biopsy confirming the diagnosis of stage IV peripheral T-Cell lymphoma. One month later, the patient presented with worsening shortness of breath. A chest radiograph revealed mild cardiomegaly and increased interstitial markings. Chest CT was notable for smooth and nodular intra- and interlobular septal thickening in lower lung fields, most consistent with lymphangitic carcinomatosis. At the time of our examination, the patient was noted to be tachycardic, tachypneic, and mildly hypoxic. Pulmonary exam revealed bibasilar rales; cardiac exam was significant for an S3 gallop, a soft systolic murmur, and jugular venous distention estimated at 12 cm H20; abdominal exam revealed splenomegaly; trace lower extremity edema was also noted. Echocardiogram demonstrated an irregularly thickened left ventricle with trabeculations, resulting in severe diastolic dysfunction; however, the systolic function was preserved. Further work-up with contrast enhanced chest CT was notable for hypodensity of the myocardium corresponding to the area of irregularity seen on the echocardiogram. This was followed by a cardiac MRI, with short Axis Phase Sensitive Inversion Recovery Delayed enhancement, showing increased signal throughout the endocardium of the left ventricle, as well as pronounced thickening of the left ventricle consistent with an infiltrative disease. The patient improved dramatically after treatment with diuresis, beta-blockers, and ACE inhibitors. Follow-up chest imaging, including repeat chest CT, showed complete resolution of the intra- and interlobular septal thickening. Furthermore, post-chemotherapy echocardiography demonstrated complete resolution of the endocardial infiltrative process.

DISCUSSION: Historically, cardiac metastases have been reported in anywhere from 8.7% to 20% of patients with lymphoma post-mortem, the vast majority of which are of B-cell origin [1,2]. Despite the development of advanced imaging modalities, this disease is seldom detected clinically. We describe a very rare presentation of T-cell lymphoma manifesting as diffuse endocardial infiltration with resultant diastolic heart failure. Dyspnea in patients with advanced cancer and interstitial infiltrates commands an extensive differential diagnosis, including a variety of infectious processes, pulmonary edema, lymphocytic interstitial pneumonia, and lymphangitic carcinomatosis. In this patient, the nodular pattern of the intra- and interlobular septal thickening was most concerning for lymphangitic carcinomatosis, particularly as some literature suggests an increased risk of pulmonary involvement in patients with endocardial infiltration of lymphoma [3]. Nonetheless, the physical exam was consistent with heart failure, prompting the appropriate investigation and diagnosis of restrictive cardiomyopathy due to endocardial invasion.

CONCLUSIONS: Cardiac involvement due to lymphoma is a rare ante-mortem finding as this disease seldom presents as heart failure. The differential diagnosis for dyspnea in a patient with advanced lymphoma and interstitial edema is broad and all potential causes must be entertained. To our knowledge, this is the first reported case of stage IV peripheral T-cell lymphoma presenting with pulmonary edema due to endocardial tumor invasion. Furthermore, the presence of delayed endocardial enhancement on cardiac MRI has never been reported in patients with cardiac involvement due to lymphoma.

Reference #1 O’Mahony D., Piekarz R., Bandettini P. et al. Cardiac involvement with lymphoma: a review of the literature. Clin Lymphoma Myeloma. 2008 August; 8(4): 249-252.

Reference #2 Giunta R., Cravero R., Granata G. et al. Primary cardiac T-cell lymphoma. Ann Hematol. 2004; 83: 450-454.

Reference #3 O’Mahony D., Debnath I., Janik J., et al. Cardiac involvement with human T-cell lymphotrophic virus type-1-associated adult T-cell leukemia/lymphoma: The NIH experience. Leuk Lymphoma. 2008 March; 49(3): 439-46.

DISCLOSURE: The following authors have nothing to disclose: Tarik Ngab, Nidhi Nikhanj, Mariam Thomas, Robin Wachsner, Nader Kamangar

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