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Review
. 2020 Sep 24;15(1):117.
doi: 10.1186/s13000-020-01034-7.

Fibrin-associated diffuse large B-cell lymphoma with plasmacytic differentiation: case report and literature review

Esther Moreno Moreno  1 , Ana Ferrer-Gómez  1 , Héctor Pian Arias  1 , Irene García García  2 , Mónica García-Cosío  3
Affiliations
Review

Fibrin-associated diffuse large B-cell lymphoma with plasmacytic differentiation: case report and literature review

Esther Moreno Moreno et al. Diagn Pathol. .

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: Case report; Epstein-Barr virus; Fibrin-associated diffuse large B-cell lymphoma; Myxoma; Primary cardiac lymphoma.

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Conflict of interest statement

The authors declare that they have no conflicts of interest.

Figures

Fig. 1
Fig. 1
a Macroscopic view of 3.5 × 3 cm solid, brownish polypoid specimen with a 2.3 × 0.7 cm pedicle. b HE (20x). Histological study revealed a proliferation composed of a dense eosinophilic substance with myxoid basophilic areas, in which spindle and starry cells were observed. No atypia or mitotic figures were identified. c HE (100x on oil). Within the myxoma, small aggregates of lymphoid-like cells were identified. High magnification showed vesicular nuclei and conspicuous nucleoli with several mitotic figures and nuclear debris. d CD79 (40x). Lymphoma cells were positive for CD79. e OCT2 (40x). Lymphoma cells were positive for OCT2. f MUM1 (40x). Lymphoma cells showed non-germinal centre phenotype, with MUM1 positivity
Fig. 2
Fig. 2
a Kappa (HIS 40x) & b Lambda (HIS 40x). Malignant cells showed kappa light chain restriction. c EBV (40x). EBERs study was positive in malignant cells. d PDL1 (40x). Programmed Death-Ligand 1 (PD-L1) study showed positivity in about 60% of the malignant cells
See this image and copyright information in PMC

References

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