Journal of Blood & Lymph

Precursor B-Cell Lymphoblastic Primary Cardiac Lymphoma – Case Report and Review of Literature

Abstract

Dipesh Uprety, Jayadev Manikkam Umakanthan, Keerthi Banda, Navin Victor, Herbert Auerbach

Primary cardiac lymphoma (PCL) is an extremely rare malignancy and an uncommon presentation of Non-Hodgkin’s Lymphoma. We report the first case of Precursor B-cell lymphoblastic PCL. A 44 year old Caucasian woman presented to our institution with one week history of worsening dyspnea, leg swelling and a weight gain of 12 pounds. Urgent echocardiography revealed pericardial effusion with tamponade physiology. Subsequent imaging showed a mass in the right atrio-ventricular groove. Pericardiocentesis was performed and cytopathology showed malignant appearing immature lymphocytes. Immunophenotypic analysis revealed 85% of the cells to be CD19 and CD10 positive. The cells were negative for CD20, surface light chains, CD3 and CD5 consistent with immature B-cells. Terminal deoxynucleotidyl Transferase (TDT) was strongly positive confirming Precursor B-cell Lymphoblastic Lymphoma. Clinical presentation of PCL can be varied and can mimic non-neoplastic primary cardiac problems. When PCL is suspected and a pericardial effusion is present, pericardial fluid analysis can be a reasonable first step. If diagnostic, it can avoid further invasive procedures. Chemotherapy remains the standard treatment and should be initiated early given the aggressive nature of these high grade lymphomas. Surgery is needed only in selected cases with obstructive features.