Title

A Rare Case Of Rapid Cardiac Invasion By Aggressive Diffuse Large B-Cell Lymphoma

Authors

Rafael Cavalcante, M.D. Gary Lu, M.D., Ph.D. Anunaya Aashish, M.D. Ana Velasquez, M.D.

Introduction

Diffuse large B-cell lymphoma, the most common subtype of non-Hodgkin lymphoma, is an AIDS-defining cancer [1]. In HIV-positive individuals, it can involve atypical sites, such as the pericardial and pleural cavities, although primary effusion lymphoma is rare [2]. Primary cardiac lymphomas are also extremely rare, their incidence is unknown, considering that not many cases have been published to date [3].

Case Presentation

We present a 52-year-old HIV-positive man, on Bictegravir, Emtricitabine and Tenofovir, who experienced recurrent cardiac and pleural effusions over a year. He initially presented at another hospital with worsening exertional dyspnea and chest pain. A chest CT showed large pericardial and minimal pleural effusions, along with mildly enlarged mediastinal lymph nodes. Pericardiocentesis and pleural fluid analysis revealed atypical large B lymphocytes, but Diffuse large B-cell lymphoma (DLBCL) was not considered at the time. He lost follow-up and was readmitted multiple times for congestive heart failure and stable angina. A cardiac MRI during another admission showed a heterogeneous pericardial lesion and asymmetric left ventricular wall thickening, when ventricular remodeling was suspected. Few months later, upon admission to our hospital with worsening symptoms, a chest CT revealed an 8.8 x 4.7 cm mass extending from the right atrium to the right ventricle and pulmonary trunk. A second mass (5.5 x 3.7 cm) was found in the basal inferolateral left ventricular wall on MRI. Heart biopsy confirmed large B-cell lymphoma, and the patient was treated with etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone and rituximab (R-CHOP). After four months, his symptoms and effusions resolved, and PET scan showed complete remission. However, five months later, exertional dyspnea returned, and imaging confirmed a large pleural effusion, with cytology showing recurrence of large B-cell lymphoma. The patient was offered treatment with CART-cell therapy, however he declined further treatment and opted for palliative care.

Discussion

This case highlights an aggressive DLBCL presenting as recurrent pericardial and pleural effusions, rapidly invading the heart and causing heart failure. Although the majority of patients with DLBCL achieve remission with R-CHOP therapy, up to 40% may experience treatment resistance or relapse following an initial response [4,5]. Anti-CD19 CART-cell therapy is currently the favored approach for the cases of high-risk DLBCL, characterized by primary refractory disease or relapse within 12 months [6]. A narrow differential diagnosis, compounded by diagnostic anchoring and availability bias, delayed the accurate identification of DLBCL, despite early indications such as the presence of atypical large B lymphocytes in the pericardiocentesis and pleural fluid analyses and later abnormal findings on cardiac MRI. Diffuse large B-cell lymphoma is highly unlikely to be initially considered the etiology of congestive heart failure unless a broad differential diagnosis is employed and there is a high index of suspicion, particularly in HIV-positive patients.

References

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