Regression of Cardiac Lymphoma With Chemotherapy

A patient was admitted for chest pain with electrocardiographic changes, and cardiac magnetic resonance showed focal myocardial hypertrophy secondary to edema. Combined positron emission tomography and computed tomography corroborated foci of myocardial hypermetabolism, as well as multiple adenopathies consistent with lymphoma in the biopsy. Hypertrophy and edema regressed with chemotherapy.

The physical examination was normal, but the electrocardiogram (ECG) (Figure 1) showed a slight elevation of the ST-segment in the inferior leads and depression of the ST-segment and a negative T-wave in leads I and aVL, without evolutionary changes in the successive ECGs.On the initial laboratory tests, she showed a high-sensitivity cardiac troponin T value of 18 ng/L (normal value [NV] <13 ng/L) with no other significant findings.

An initial bedside echocardiogram (Videos 1 to 3)
showed preserved biventricular ejection fraction, although mild focal hypokinesia and thickening of the basal anterolateral segment were detected.

PAST MEDICAL HISTORY
The patient had no previous medical history or cardiovascular risk factors.However, she reported a previous history of 3 months of limb polyarthralgias and migratory dysesthesias.

LEARNING OBJECTIVES
To be able to make a differential diagnosis of myocardial injury with multimodality imaging.To keep in mind extracardiac causes of myocardial injury.To correlate the different imaging modalities and their usefulness.

DIFFERENTIAL DIAGNOSIS
Given the recurrence of chest pain with a slightly elevated troponin level and the ECG findings, ST-segment elevation myocardial infarction was the primary working diagnosis.Therefore, emergency invasive coronary angiography was performed, and it showed no significant coronary artery disease.A thoracic computed tomography (CT) scan (Figure 2) was also performed to rule out other life-threatening causes of intense chest pain, such as acute aortic syndrome or pulmonary embolism, and it revealed several small axillar and mediastinal adenopathies.The patient was admitted to the cardiology ward.The scan revealed multiple supradiaphragmatic and  The image shows several small axillary and mediastinal adenopathies (arrows).Other presentations of lymphoproliferative processes in the heart are primary tumors, where lymphomas represent just 10% of primary malignant heart tumors and approximately 1% of all primary cardiac tumors.CMR also has a key role in this entity.

INVESTIGATIONS
Primary cardiac lymphomas more frequently involve the right side of the heart and usually manifest with right-sided congestive heart failure. 6 our patient, acute chest pain was the first guiding symptom that led to the final (and relatively early) diagnosis.Unfortunately, given the severity of the hematologic disease, her prognosis was eventually equally poor.The imaging shows high extracellular volume on the anterior segment (blue, normal extracellular volume; green, high extracellular volume).The imaging reveals an intramyocardial heterogeneous and faint contrast medium retention in the hypertrophic segments (arrows).

FUNDING SUPPORT AND AUTHOR DISCLOSURES
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.E-mail: jfernandezma@santpau.cat.@juanfm_MD.The scan shows polyadenopathies (red arrows) and myocardial hypermetabolic foci (white arrows).Fernández-Martínez et al Initial cardiac magnetic resonance (CMR) was performed, with a 1.5-T scan showing a nondilated left ventricle with preserved ejection fraction but with significant thickening of the basal and midanterior and anterolateral segments (maximal 17 mm) (Video 4).Those segments showed high T2 (>65 ms; NV <55 ms) and T1 (1,230 ms; NV <1,050 ms) mapping values (Figures 3 and 4), with an increased extracellular volume of 33% (NV<30%), suggesting the presence of abundant myocardial edema.Late gadolinium enhancement sequences revealed an intramyocardial heterogeneous and faint contrast medium retention in the same hypertrophic segments (Figure 5).The report concluded with a probable diagnosis of myocardial pseudohypertrophy secondary to important myocardial inflammation of unknown origin.Because myocarditis was suspected, antiinflammatory treatment was started, and the patient achieved complete remission of chest pain.Laboratory tests were amplified with viral serologic studies (SARS-CoV-2, Epstein-Barr, and parvovirus B19) and a complete rheumatologic profile, all of which had negative results.The proteinogram revealed an isolated elevation of b 2 -microglobulin of 3.38 mg/L (NV <1.8 mg/L).The presence of adenopathies on the thoracic CT scan raised the suspicion of possible underlying cardiac inflammatory disease.Therefore, the patient underwent a positron emission tomography (PET)/ CT scan with fluorine-18 fluorodeoxyglucose (FDG).

FIGURE 1
FIGURE 1 Electrocardiogram on Arrival

FIGURE 2
FIGURE 2 Computed Tomography Scan

FIGURE 3 T2
FIGURE 3 T2 Mapping on the Initial Cardiac Magnetic Resonance

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A C C : C A S E R E P O R T S , V O L . 2 9 , 2 0 2 4Fernández-Martínez et al such as arrhythmias 2 or heart failure 3 ; chest pain is not a common first presentation.Secondary cardiac involvement from lymphoma is a relatively frequent occurrence, reported in up to 25% of patients 4 ; this incidence is probably underestimated because of a high percentage of asymptomatic cases.Myocardial involvement of adult T-cell leukemia/lymphoma is often detected during autopsy; however, the development of cardiac symptoms is extremely rare, with only 7 registered cases.5

FIGURE 4
FIGURE 4 Extracellular Volume Map on the Initial Cardiac Magnetic Resonance

FIGURE 5
FIGURE 5 Late Gadolinium Enhancement Sequences on the Initial Cardiac Magnetic Resonance

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A C C : C A S E R E P O R T S , V O L . 2 9 , 2 0 2 4 Cardiac Lymphoma Regression With Chemotherapy FOLLOW-UP Follow-up CMR was performed, and it showed preserved biventricular function with resolution of myocardial edema (normalization of the T1 and T2 mapping values) and regression to normal wall thickness, leaving residual soft myocardial fibrosis in the previously inflamed segments (Figure 8).The subsequent FDG PET/CT scan confirmed the absence of uptake at the myocardial level despite systemic progression of the lymphoproliferative process (Figure 8).Finally, a third line of chemotherapy was started without neurological improvement.Despite an excellent documented cardiac response, she presented with refractory cranial hypertension, which led to a fatal outcome 9 months after the initial diagnosis.CONCLUSIONS Myocardial injury is usually a diagnostic challenge that is not always possible to resolve.The advancement of different multimodal imaging techniques allows us to obtain more information, leading to increasingly precise and complete diagnoses.It is a difficult scenario, and often the cause is not primarily cardiac, so it is important to keep an open and objective mind.

FIGURE 7
FIGURE 7 Biopsy of an Inguinal Node

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A C C : C A S E R E P O R T S , V O L . 2 9 , 2 0 2 4