Background

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma, with an increased risk associated with Human Immunodeficiency Virus (HIV) infection. In the United States, Burkitt lymphoma often involves intra-abdominal organs or lymph nodes. Here, we present an interesting and exceedingly rare case of Burkitt lymphoma involving the heart in an HIV patient.

Case Presentation

A 40-year-old male presented with a newly noticed swelling in his right axilla. He reported a 40-pound weight loss along with occasional fever, night sweats, and abdominal pain. Physical examination revealed a 4 cm subcutaneous mass without skin changes. Vital signs and initial lab results were unremarkable. CT scans of the chest, abdomen, and pelvis revealed a 7-cm right axillary mass, marked diffuse thickening of the gastric and small bowel walls, and a large right atrial filling defect. An excisional biopsy of the axillary mass confirmed Burkitt lymphoma. HIV serology was positive, with a viral load of 229,000 copies/ml, and LDH was elevated at 503 U/L. Peripheral blood smear showed no atypical cells, and bone marrow aspiration, biopsy, and flow cytometry were negative for lymphoma involvement. The patient was diagnosed with stage IIIBE Burkitt lymphoma and started on chemotherapy, including cyclophosphamide, doxorubicin, vincristine, methotrexate, ifosfamide, etoposide, and cytarabine.

After one month, a follow-up CT scan showed a significant reduction in the size of the right axillary and atrial masses, as well as in the thickening of the small bowel and gastric walls. Given the significant treatment response of the intracardiac mass, the diagnosis was confirmed as Burkitt lymphoma with intracardiac involvement.

Discussion

Intracardiac Burkitt lymphoma is an extremely rare malignancy, with only 22 reported cases to date. As with other forms of Burkitt lymphoma, HIV infection increases the risk of developing intracardiac Burkitt lymphoma. The symptoms are often nonspecific and cardiopulmonary symptoms such as palpitations or dyspnea can occur in other types of Burkitt lymphoma as well.

Given the highly aggressive nature of intracardiac Burkitt lymphoma and its potential to cause arrhythmias or pericardial effusion, which can lead to life-threatening conditions, early diagnosis is crucial. However, the extreme rarity of the disease and the nonspecific nature of its symptoms often lead to a low level of awareness among physicians, increasing the likelihood of missed diagnoses. Therefore, it is essential for physicians to maintain a high index of suspicion for intracardiac involvement when diagnosing Burkitt lymphoma.