Fibrin-associated diffuse large B-cell lymphoma with plasmacytic differentiation: case report and literature review
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CASE REPORT
Open Access
Fibrin-associated diffuse large B-cell lymphoma with plasmacytic differentiation: case report and literature review Esther Moreno Moreno1, Ana Ferrer-Gómez1, Héctor Pian Arias1, Irene García García2 and Mónica García-Cosío1*
Abstract Background: Primary cardiac lymphomas are extremely rare entities (< 2% of cardiac tumours) and the most frequent histologic type is diffuse large B-cell lymphoma (DLBCL). Fibrin-associated DLBCL (FA-DLBCL) is a very unusual form of DLBCL associated with chronic inflammation, and only case reports and small series have been described. In the heart, it usually occurs in the context of a cardiac myxoma or cardiac prostheses and it is not bulk forming. These lymphomas frequently present with non-germinal center phenotype and are associated with Epstein-Barr virus (EBV) type III latency. Case presentation: We describe a case of FA-DLBCL arising in a cardiac myxoma, with plasmacytic differentiation and type I EBV latency. Conclusions: Although they are very rare, FA-DLBCLs should be known for their diagnostic difficulty, due to its unspecified clinical manifestations, and for their more favourable prognosis, sometimes even without additional treatment after surgical resection. Keywords: Myxoma, Primary cardiac lymphoma, Fibrin-associated diffuse large B-cell lymphoma, Epstein-Barr virus, Case report
Background Primary cardiac lymphomas (PCLs) are extremely rare (< 2% of cardiac tumours) [1, 2]. The most frequent histologic type is diffuse large B-cell lymphoma (DLBCL) [1]. Conventional PCLs, such as DLBCL, infiltrate myocardium or pericardium, with aggressive course and low median survival [2]. DLBCL associated with chronic inflammation (DLBCL-CI) occurs in the context of a localized and maintained situation of chronic inflammation and shows association with Epstein-Barr virus (EBV) [3]. The most representative example within this group is pyothorax-associated lymphoma [4]. Fibrin-associated DLBCL (FA-DLBCL) is a very unusual form of this * Correspondence: [email protected] 1 Department of Pathology, Hospital Universitario Ramón y Cajal, Ctra. de Colmenar Viejo km 9,100, Madrid 28034, Spain Full list of author information is available at the end of the article
entity, which is mediated by a local immunosuppression mechanism and can be found associated to cardiac myxoma or cardiac prostheses [1]. In FA-DLBCLs, tumour cells are arranged singly or in small clusters within fibrin material [2] or associated to chronic blood effusions [5], with a high proliferative index (Ki-67 > 90%), but without myocardial invasion [1]. The available literature about this entity is scarce and only case reports or small series have been published. These lymphomas usually present with non-germinal center phenotype and associated with EBV type III latency, having better prognosis than conventional DLBCL and DLBCL-CI [2]. We report a case of FA-DLBCL with plasmacytic differentiation and associated to EBV type I latency, arising in a cardiac myxoma and growing in small
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