Cardiac lymphoma requiring urgent heart transplant due to ventricular tachycardia storm: a case report.

BACKGROUND: Primary cardiac lymphoma (PCL) involves the heart almost exclusively although it can extend to surrounding structures including the pericardium. Most PCLs in adults are of B-cell origin and their signs and symptoms are generally non-specific and depend on their location and size. In general, cancer patients usually have a slim chance of receiving heart transplantation (OHT), although it's not an absolute contraindication depending on the decision of the multidisciplinary team and the experience of each institution.

CASE SUMMARY: A 48-year-old man, with an ultimate diagnosis of primary cardiac follicular B-cell lymphoma presented to our hospital mimicking hypertrophic cardiomyopathy. He initially presented with worsening heart failure and ventricular tachycardia storm (VT-S) that required urgent cardiac OHT. The final pathological analysis of the explanted heart revealed the presence of a PCL without extra-cardiac extension. In addition to initial immunosuppression with mycophenolate mophethyl, corticosteroids, and tacrolimus, he was switched to Everolimus and dose reduction of Tacrolimus. Rituximab + Bendamustine was initiated to reduce the risk of cardiotoxicity and myelotoxicity associated to R-CHOP. The follow-up body PET-CT, trans-thoracic echocardiogram, cardiac magnetic resonance imagings and biopsies were normal. During a regular follow-up heart biopsy procedure to ascertain rejection, the patient developed torrential tricuspid regurgitation and required surgical valve replacement.

DISCUSSION: After 5.5 years of follow-up, the patient remains asymptomatic, with normal graft function, in NYHA FC I, and without oncological relapses despite receiving a modified chemotherapy regimen. Selected patients with a PCL can be managed with OHT and a modified chemotherapy regimen. They could also be followed up using a non-invasive approach to monitor rejection.