A Rare Case of Burkitt’s Lymphoma Presenting as Mixed Constrictive and Obstructive Cardiogenic Shock

Abstract

Burkitt’s lymphoma is a highly aggressive B-cell lymphoma that rarely presents as an intracardiac mass or extracardiac mass involving the pericardium. We report a case of extracardiac Burkitt’s lymphoma in a previously healthy 59-year-old man who presented with mixed constrictive and obstructive cardiogenic shock. Diagnosis was facilitated through multimodality imaging, including transthoracic echocardiography, cardiac MRI, chest CT, and invasive hemodynamic assessment using pulmonary artery catheters. These tools were instrumental in characterizing the constrictive and obstructive components of the patient’s shock. A pulmonary artery catheter was also employed for ongoing hemodynamic monitoring during treatment with chemotherapy and tumor debulking agents. Remarkably, the patient showed rapid clinical improvement within 48 hours of initiating therapy, as evidenced by the hemodynamic monitoring trends.

Case Presentation

A 59-year-old man with a medical history of hypertension and a 17-pack-per-year smoking history presented to an outside hospital after a febrile illness with progressively worsening dyspnea, fatigue, and a 2-day history of night sweats. During this initial admission, the patient was in atrial flutter with 2:1 conduction and was planned for a cardioversion. Transesophageal echocardiogram revealed a left atrial appendage thrombus and a thickened pericardium. Cardioversion was deferred due to the presence of the thrombus, and the patient was discharged on metoprolol succinate and apixaban. He presented again several days later to the same outside hospital with a new oxygen requirement and a 10-pound weight gain.

The patient underwent an initial right heart catheterization, which demonstrated a right atrial pressure (RAP) of 17 mmHg, pulmonary artery pressure of 32/20 mmHg with a mean of 24 mmHg, pulmonary capillary wedge pressure (PCWP) of 18 mmHg, pulmonary artery saturation of 39%, and an assumed Fick CI of 1.3 l/min/m². Additional imaging included cardiac MRI, which revealed a small-to-moderate pleural effusion, pericardial thickening, mass encasing the right atrium, and a mediastinal mass with late gadolinium enhancement concerning for an infiltrative process (Figure 1). Chest CT revealed large bilateral pleural effusions (Figure 2), and subsequent thoracentesis revealed an exudative process. Based on these findings, the patient was transferred to a large academic center for further management of cardiogenic shock.

Figure 1: Cardiac MRI Showing Mass Encasing the Right Atrium, Pericardial Thickening and Effusions.

Late gadolinium enhancement sequence (motion-corrected phase-sensitive inversion recovery) of the four-chamber (A) and three-chamber (B) demonstrating patchy uptake of the right atrial mass (arrow), pericardial thickening and mediastinal mass surrounding the aortic root and superior vena cava (star). Steady state free precession four-chamber (C) and short axis (D) demonstrating right atrial mass at the junction of the inferior vena cava (arrow), thickening of the pericardium (star), small-to-moderate pericardial effusion and bilateral pleural effusions.

Figure 2: CT Chest Showing Mediastinal Mass, Pericardial Thickening, Effusions.

Coronal view (A) and axial stack (B and C) of CT chest showing pericardial thickening, complex pericardial effusion, mediastinal mass surrounding the aorta, and bilateral pleural effusions. The arrow shows a mass encroaching on the superior vena cava.

Investigations

On presentation to our facility, the patient was afebrile with a blood pressure of 117/80 mmHg, mild tachycardia with a heart rate of 103 BPM, respiratory rate of 22 breaths/min, and oxygen saturation of 96% on 4 l nasal cannula. Initial diagnostics included a transthoracic echocardiography, which demonstrated normal left ventricular size and a left ventricular ejection fraction of 65% with mild concentric left ventricular hypertrophy. A small pericardial effusion was noted with respiratory variation of mitral inflow <30%, respiratory variation of tricuspid inflow <50%, and no evidence of tamponade. There was also evidence of a respirophasic septal bounce and mitral annulus reversus. Pericardial thickening was particularly pronounced along the right ventricular free wall, with apparent tethering to both the lateral mitral annulus and the right ventricular free wall (Figure 3). A dilated and non-collapsible inferior vena cava was also presented, suggestive of an elevated RAP.

Figure 3: Echocardiography Showing Right Ventricular Pericardial Thickening and Effusion.

Parasternal short axis (A) and apical four-chamber (B) demonstrating pericardial thickening (star) of the right ventricle with small pericardial effusion.

The patient underwent a second right heart catheterization, which demonstrated a RAP of 15 mmHg, pulmonary artery pressure of 30/20 mmHg, with a mean of 23 mmHg, PCWP of 17 mmHg, and an assumed Fick CI of 1.5 l/min/m². There was also subtle evidence of ventricular interdependence (Figure 4). There was some difficulty getting into the right atrium, requiring a wire. Repeat chest CT showed evolving complex pericardial effusion and pericardial thickening, which was concerning for a mediastinal mass encasing the great vessels. CT of the abdomen and pelvis was notable for a short segment of circumferential wall thickening in the small bowel and adjacent soft tissue stranding and subtle soft tissue nodularity, thought to be an acute inflammatory process or possible underlying malignancy.

Figure 4: Catheterization Tracing Demonstrating Interventricular Dependence.

Right heart catheterization tracing with arrow pointing to evidence of interventricular dependence at the sixth beat with end expiration.

The differential diagnosis was broad, including infectious, inflammatory and malignant etiologies. The recent febrile illness was reported by the patient to be COVID-19. A broad infectious differential was considered due to the pericardial effusion, which included coxsackievirus A/B, echovirus, adenovirus, Epstein-Barr virus, cytomegalovirus, HIV, and hepatitis B. Bacterial and fungal etiologies were also on the differential, given the presence of exudative pleural effusions reported at the outside hospital and because the patient worked as a florist, although he was not known to be immunocompromised. Inflammatory conditions were felt to be less likely without a history of arthralgias, rash, or positive family history. Lastly, malignant causes were also strongly considered given the patient’s smoking history, family history of lung cancer (his father), as well as elevated lactate dehydrogenase, and early evidence of hyperkalemia and hyperuricemia, concerning for tumor lysis syndrome.

The patient underwent upper hemisternotomy with pericardial biopsy and subtotal excision of the mediastinal mass. Operative findings included a thickened pericardium and a white pearly mass between the aorta and superior vena cava. Thoracentesis was performed with pleural fluid cytology and mediastinal biopsy pathology revealing an aggressive large B-cell lymphoma, MYC:IGH rearrangement positive, Ki-67 >95%, numerous tingible body macrophages with starry sky appearance, consistent with Burkitt’s lymphoma. A PET with fludeoxyglucose (FDG) demonstrated widespread lymphomatous involvement of all visualized marrow space elements, pericardial and pleural surfaces, non-enlarged upper abdominal lymph nodes and visceral involvement of the spleen and left lower quadrant bowel loops, consistent with a Deauville score of 5 (Figure 5).

Figure 5: EKGs Throughout Hospital Course.

A: EKG with atrial tachycardia. B: EKG with junctional rhythm after amiodarone therapy.

The patient was treated with dobutamine and furosemide infusions. PCWP was used to gauge diuresis as the RAP was unreliable due to compression by the residual mediastinal mass on the superior vena cava. There were discrepancies between assumed Fick and thermodilution, with Fick demonstrating a CI of 1.5 l/min/m² and simultaneous thermodilution suggesting a consistently higher CI of 2–2.5 l/min/m² prior to the onset of chemotherapy. Thermodilution cardiac indices were continued for hemodynamic monitoring as they were considered more reliable given the catabolic state and increased metabolic demand from the underlying malignancy, which would not be taken into account by the estimated oxygen consumption (Dehmer estimate) in the assumed Fick equation. The patient was maintained on a heparin infusion for known left atrial appendage thrombus as well as persistent atrial tachycardia. He was treated with amiodarone, resulting in a junctional rhythm on EKG (Figure 5), which may have been caused by sinus node compression from the mass versus postoperative edema in that region.

Notably, research has indicated that patients with lymphoma affecting the heart, even without a defined intra- or extra-cardiac mass, can present with symptoms such as dyspnea, tachycardia, and arrhythmias visible on EKGs. This further highlights the importance of hemodynamic data and using multiple imaging techniques, as was done in this case.1

Upon confirmation ofthe diagnosis of Burkitt’s lymphoma, the patient was started on dose-adjusted cyclophosphamide, etoposide, doxorubicin, prednisone, and rituximab (DA-EPOCH-R) in addition to intrathecal methotrexate and hydrocortisone. Within 48 hours of starting chemotherapy, he was weaned from dobutamine and maintained a CI of 2.5 l/min/m² off inotropic support, evidence of rapid debulking and improvement in mixed constrictive/obstructive shock with appropriate therapy, as further evidenced by interval improvement in repeat PET-FDG scan (Figure 6).

Figure 6: PET Showing Fludeoxyglucose Uptake with Interval Improvement after Therapy.

A: Schematic and PET imaging demonstrating obstructive and constrictive pathology. B: Fludeoxyglucose-avid regions within the pericardium and surrounding the ascending aorta and superior vena cava. C: Interval improvement in fludeoxyglucose uptake following therapy.

Discussion

Diagnosis was particularly challenging due to the coexistence of both constrictive and obstructive physiologies, each capable of leading to decreased cardiac output. Constrictive physiology usually manifests with increased RAP and rapid and limited ventricular filling due to a stiff, non-compliant pericardium. Notably, evidence of a respirophasic septal bounce and mitral annulus reversus was present in our patient, findings commonly associated with constrictive physiology.

Mitral annulus reversus is characterized by a reversal of the normal relationship between the medial and lateral mitral annular velocities.2 In typical physiology, the lateral mitral annulus exhibits higher early diastolic velocities compared to the medial annulus; however, patients with constrictive physiology often demonstrate the inverse relationship.2 This phenomenon reflects the mechanical restrictions imposed by a thickened pericardium, which inhibits lateral wall expansion and alters diastolic function.2 Additionally, septal bounce, which is an abnormal diastolic motion of the interventricular septum, was observed, a finding with a reported sensitivity of 62% and specificity of 93% for constriction.3

Furthermore, right heart catheterization revealed some degree of ventricular interdependence; it was only observed transiently, specifically at the sixth beat at end expiration (Figure 4), which complicated the interpretation. Notably, the presence of arrhythmia at the time may have interfered with the accurate assessment of ventricular interdependence. Ultimately, malignant pericardial infiltration led to pericardial constriction and a low-output state, while an extracardiac mass compressing the superior vena cava and right atrium contributed to obstructive physiology (Table 1 and Figure 6). Together, these factors produced a mixed shock, necessitating both careful management of cardiac filling pressures and inotropic support. Although PA catheter use in heart failure patients has declined, recent studies have shown improved survival in cardiogenic shock patients when PA catheters were employed. A PA catheter was essential for monitoring our patient’s tenuous state and instrumental in observing the significant improvement following initiation of appropriate therapy.4

Table 1: Imaging Modality and Diagnostic Findings.

Imaging Modality	Constrictive Physiology	Obstructive Physiology
Chest CT	Complex pericardial effusion and pericardial thickening	Mediastinal mass encasing the great vessels leading to venous narrowing
Transthoracic echocardiogram	Pericardial effusion with respiratory variation of mitral inflow <30% Respirophasic septal bounce and mitral annulus reversus Pericardial thickening and tethering to the lateral mitral annulus and the right ventricular free wall	Dilated and non-collapsible inferior vena cava
Cardiac MRI	Pericardial thickening	Mediastinal mass encasing the great vessels leading to venous narrowing
Pulmonary artery catheter	Mild ventricular interdependence	Difficulty to thread wire during right heart catheterization

Fortunately, Burkitt’s lymphoma is highly responsive to intensive chemotherapy, with a 4-year overall survival rate of 84% following DA-EPOCH-R, and often responds quickly to chemotherapy as evidenced by the development of tumor lysis syndrome.5 Burkitt’s lymphoma involving intracardiac or extracardiac cardiac masses affecting the pericardium is exceedingly rare. Among 197 reported cases of primary cardiac lymphoma from 1949 to 2009, only 13 (6.6%) were Burkitt’s lymphoma.6 Additional clues suggestive of possible underlying malignancy included the development of hyperkalemia and hyperuricemia prior to the onset of chemotherapy, suggesting spontaneous tumor lysis syndrome.

The patient remained off inotropes and hemodynamically stable. He was then transferred to the oncology unit and completed his first cycle of DA-EPOCH-R as an inpatient prior to discharge. His course was complicated by sepsis and mucositis, but he remained hemodynamically stable without evidence of recurrent shock. He was ultimately discharged with close oncology follow-up.

Conclusion

The present case emphasizes the utility of invasively measured hemodynamics via a pulmonary artery catheter, which allowed for quick diagnosis of mixed constrictive and obstructive cardiogenic shock. Multimodality imaging, including transthoracic echocardiography, cardiac MRI, and CT chest scans, demonstrated evidence of a malignant process, which was confirmed on fluid cytology and tissue biopsy. Prompt initiation of chemotherapy and debulking agents resulted in quick resolution of shock, as evidenced by PA catheter hemodynamic trends. This case demonstrates how a PA catheter provided real-time monitoring of hemodynamic improvement after chemotherapy, suggesting its value for similar challenging diagnoses.

Clinical Perspective

• Burkitt’s lymphoma presenting as a cardiac mass is extremely rare.

• Multimodality imaging, including transthoracic echocardiography, cardiac MRI, and CT provided critical information to aid in the diagnosis of mixed constrictive and obstructive physiology.

• Invasive hemodynamics monitoring via pulmonary artery catheter played a vital role in the diagnosis of constrictive and obstructive cardiogenic shock, as well as in monitoring the patient’s response to treatment.

• This case highlights the rapid reversal of cardiogenic shock following the initiation of chemotherapy and tumor debulking agents, as demonstrated by significant improvements in pulmonary artery catheter-derived hemodynamic parameters.