Epstein-Barr Virus-associated Diffuse Large B-cell Lymphoma Arising on Cardiac Prostheses

Abstract

Primary cardiac lymphoma is extremely rare and lymphoma arising in association with prosthetic valves has been described in only 3 case reports. We describe 3 patients with diffuse large B-cell lymphoma (DLBCL) involving prosthetic heart valves and a synthetic tube graft. All 3 specimens showed shallow layering of acellular fibrinous debris over the prosthetic or synthetic materials, with tumor lymphocytes present at the luminal surface. There were frequent mitoses and abundant karyorrhectic debris. All demonstrated a nongerminal center B-cell phenotype. All 3 cases were positive for Epstein-Barr virus, but there was no staining for human herpes virus 8. There was no other evidence of distant disease at the time of diagnosis and no recurrence or dissemination occurred after surgical removal of the prosthesis, though follow-up was limited. On the basis of 2008 World Health Organization diagnostic criteria, we believe these cases should be classified as DLBCL associated with chronic inflammation. However, unlike the characteristically poor prognosis reported in this entity, we hypothesize that the disease resectability in these cardiac sites, in many cases, may allow for a better prognosis than DLBCL with chronic inflammation at other less resectable sites.

Recurrent Primary Cardiac Lymphoma on Aortic Valve Allograft: Implications for Therapy

“…To our knowledge, ours is the 7th report of B-cell lymphoma that involved valvular prostheses (Table I), 1,[4][5][6][7] and the first involving recurrent DLBCL on an aortic allograft. All the patients listed in Table I had isolated primary cardiac lymphoma involving a prosthesis, but without disseminated lymphoma.…”

“…9 Of the 6 previous patients who had DLBCL in association with a cardiac prosthesis, 3 had mechanical valves (latency periods, 8, 9, and 24 yr), 2 had xenografts (latency periods, 3 and 8 yr), and one had an allograft (latency period unknown). 6 Our patient was given an allograft twice, because of the presence or suspicion of bacterial …”

“…12 Two patients were living without evidence of lymphoma at 6 months 1,6 ; however, no followup information was reported, so their final outcomes …”

The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications

“…In addition to lymphomatoid granulomatosis, the WHO now includes EBVpositive DLBCL of the elderly initially described in Asia [39][40][41][42] and DLBCL associated with chronic inflammation, most often chronic pyothorax. [43][44][45][46] In these lesions EBV-infected cells frequently express a latency type 3 with positivity for the viral proteins latent membrane protein 1 and EBV nuclear antigen 2, suggesting that the lymphoid proliferation may be related primarily to a decrease in the host immunosurveillance. These cases typically have a rich inflammatory background but are excluded from THRLBCL.…”

New developments in the pathology of malignant lymphoma: a review of the literature published from October 2009 to January 2010

“…The authors propose EBV-positive mucocutaneous ulcer as a newly recognized clinicopathologic entity with Hodgkin-like features and a self-limited, indolent course, generally responding well to conservative management. Somewhat different, but likely in the same spectrum are three EBVpositive lymphoproliferations described by Miller et al [53] referred to as DLBCL involving prosthetic heart valves and a synthetic tube graft. These patients had no distant disease and no recurrence after removal.…”