fibrin and Epstein-Barr-Virus-Infections

Fibrin-associated large B-cell lymphoma is a rare microscopic-sized tumor, typically representing an unexpected finding at sites rich in chronic fibrin deposition. It is associated with Epstein-Barr virus, and has been reported to occur in a wide variety of anatomic sites and clinical scenarios. We report a case arising in a thyroid hyperplastic nodule, only the second case reported in this location. Notably, this is only the fourth case of fibrin-associated large B-cell lymphoma that is not associated with Epstein-Barr virus. We provide a literature review on the clinico-pathological characteristics and outcome of this newly characterized indolent lymphoma type, which has only recently been separated out from the pathologically similar but highly aggressive large B-cell lymphoma associated with chronic inflammation.

Topics: Epstein-Barr Virus Infections; Fibrin; Herpesvirus 4, Human; Humans; Hyperplasia; Lymphoma, Large B-Cell, Diffuse; Thyroid Nodule

Topics: Atrial Fibrillation; Epstein-Barr Virus Infections; Fibrin; Herpesvirus 4, Human; Humans; In Situ Hybridization, Fluorescence; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Myxoma; Proto-Oncogene Proteins c-bcl-2; Proto-Oncogene Proteins c-bcl-6

Fibrin-associated diffuse large B-cell lymphoma (fibrin-associated DLBCL) is a very rare subtype of Epstein-Barr virus (EBV)-associated DLBCL that usually develops within fibrin-rich lesions such as cardiac myxoma, chronic hematoma, thrombus, pseudocysts or cysts, prosthetic devices or breast implants. The pathogenesis as well as the clinicopathologic features of this usually indolent lymphoproliferative disorder, which are based on the analysis of a relatively limited number of cases, are still poorly known. Herein, we report a case of fibrin-associated DLBCL that was incidentally found within a multinodular goiter, a location not yet reported to our knowledge. Our findings not only illustrate further the specific nature of this peculiar lymphoproliferative disorder but also allow some interesting comments on its pathogenesis.

Topics: Epstein-Barr Virus Infections; Fibrin; Goiter; Herpesvirus 4, Human; Humans; Lymphoma, Large B-Cell, Diffuse

Topics: Breast Implants; Epstein-Barr Virus Infections; Fibrin; Herpesvirus 4, Human; Humans; Incidental Findings; Inflammation; Lymphoma, Large B-Cell, Diffuse

Topics: Epstein-Barr Virus Infections; Fibrin; Hematoma; Herpesvirus 4, Human; Humans; Lymphoma, Large B-Cell, Diffuse; Lymphoproliferative Disorders; T-Lymphocytes

Fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is a rare Epstein-Barr virus (EBV) positive lymphoproliferative disorder included in the current World Health Organization (WHO) classification. It arises within fibrinous material in the context of hematomas, pseudocysts, cardiac myxoma or in relation with prosthetic devices. In these clinical settings the diagnosis requires an high index of suspicion, because it does not form a mass itself, being composed of small foci of neoplastic cells. Despite overlapping features with diffuse large B-cell lymphoma associated with chronic inflammation, it deserves a separate classification, being not mass-forming and often following an indolent course.. A 64-year-old immunocompetent woman required medical care for cerebral hemorrhage. Computed Tomography (CT) angiography identified an aneurysm in the left middle cerebral artery. A FA-DLBCL was incidentally identified within thrombotic material in the context of the arterial aneurysm. After surgical removal, it followed a benign course with no further treatment.. The current case represents the first report of FA-DLBCL identified in a cerebral artery aneurysm, expanding the clinicopathologic spectrum of this rare entity. A complete literature review is additionally made.

Topics: Biopsy; Computed Tomography Angiography; Epstein-Barr Virus Infections; Female; Fibrin; Herpesvirus 4, Human; Humans; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged

Incidental cases of localized fibrin-associated Epstein-Barr virus (EBV)+ large B-cell proliferations have been described at unusual anatomic sites and have been included in the category of diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) in the WHO Classification. We describe 12 cases and review the literature to define their clinicopathologic spectrum and compare features with typical cases of DLBCL-CI. Median age was 55.5 years with a M:F ratio of 3. In all 12 cases, the lymphoma was an incidental microscopic finding involving atrial myxomas (n=3), thrombi associated with endovascular grafts (n=3), chronic hematomas (n=2), and pseudocysts (n=4). All cases tested were nongerminal center B-cell origin, type III EBV latency, and were negative for MYC rearrangements and alternative lengthening of telomeres by FISH. Most showed high CD30, Ki67, and PD-L1, and low to moderate MYC and p53 expression. Among 11 patients with detailed follow-up, 6 were treated surgically, 3 with cardiac or vascular lesions had persistent/recurrent disease at intravascular sites, and 4 died of causes not directly attributable to lymphoma. Reports of previously published fibrin-associated cases showed similar features, whereas traditional DLBCL-CI cases with a mass lesion had significantly higher lymphoma-associated mortality. Fibrin-associated EBV+ large B-cell lymphoma is clinicopathologically distinct from DLBCL-CI, warranting separate classification. Most cases, particularly those associated with pseudocysts, behave indolently with the potential for cure by surgery alone and may represent a form of EBV+ lymphoproliferative disease rather than lymphoma. However, primary cardiac or vascular disease may have a higher risk of recurrence despite systemic chemotherapy.

Topics: Adult; Aged; Biomarkers, Tumor; Case-Control Studies; Chronic Disease; Epstein-Barr Virus Infections; Female; Fibrin; Follow-Up Studies; Humans; Immunohistochemistry; Inflammation; Lymphoma, B-Cell; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Prognosis

Cardiac lymphomas are rare, and the spectrum of pathologic features is not well defined. We encountered an unusual case of cardiac lymphoma residing within a presumed thrombus. To place such cases in context, we reviewed all cardiac lymphomas presenting to a large US cardiovascular medicine referral center during a 30-year period. A total of 14 cardiac lymphomas were identified, and these included 6 primary cardiac lymphomas (PCLs) and 8 lymphomas secondarily involving cardiac structures. Upon review, 3 of the PCLs were confirmed to be diffuse large B-cell lymphoma, not otherwise specified, involving the myocardium. The other 3 cases of PCL lacked myocardial invasion and showed lymphoma cells embedded in fibrin thrombus. Acute inflammation was not evident. These lymphomas presented in immunocompetent male individuals and involved either a prolapsed myxomatous mitral valve, a pseudomyxoma from the left atrium, or a thrombus arising in a synthetic aortic root graft. All 3 consisted of large atypical lymphocytes expressing a nongerminal center B-cell immunophenotype. Two cases were positive for Epstein-Barr virus (latency type III), but none demonstrated human herpes virus-8 latent nuclear antigen. No systemic disease was found at presentation or during follow-up. In our experience, fibrin-associated large B-cell lymphoma arising in the heart represents a substantial proportion of PCL. These lymphomas appear to represent an underrecognized variant of diffuse large B-cell lymphoma with favorable outcome. Further study is needed to understand their natural history.

Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Coronary Thrombosis; Epstein-Barr Virus Infections; Female; Fibrin; Heart Neoplasms; Herpesvirus 4, Human; Humans; Immunocompetence; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Myocardium; Treatment Outcome

Topics: Adult; Bone Marrow Diseases; Epstein-Barr Virus Infections; Female; Fibrin; Granuloma; Humans

We previously reported 24 cases of marrow fibrin-ring granuloma (FRG) encountered in 1 institution and concluded that, contrary to previous studies showing marrow FRG as a diagnostic marker for Q fever, Epstein-Barr virus (EBV) was the most common proven cause of such FRG. The present study characterized patients with EBV-associated marrow FRG. We retrospectively reviewed 17 cases of EBV-associated FRG (43% of 40 cases with marrow FRG) diagnosed by bone marrow biopsy. Patients with EBV-associated hemophagocytic lymphohistiocytosis (5/17 patients) and chronic active EBV infection (4/17) constituted 53% of patients with EBV-associated FRG. Three patients had lymphoma without marrow involvement. All patients except 1 presented first with fever; splenomegaly was the next most common clinical finding (12/17). Cytopenia was observed in the 17 cases: anemia in 16, thrombocytopenia in 9, and leukopenia in 8. Patients with EBV-associated FRG showed lower survival outcomes than did patients without EBV (median, 3.0 vs 11.8 months; P = .009). Patients with bone marrow FRG accompanied by fever require careful evaluation to discern whether active EBV infection is involved because the prognosis is poor.

Topics: Adult; Aged; Bone Marrow Diseases; Child; Child, Preschool; Epstein-Barr Virus Infections; Female; Fibrin; Granuloma; Humans; Lymphohistiocytosis, Hemophagocytic; Male; Middle Aged; Prognosis

Fibrin-ring granuloma (FRG), which can be found in bone marrow or the liver, is a subtype of epithelioid granuloma characterized by a central fat vacuole and annular peripheral fibrinoid materials. FRG has been proven to be associated with many etiologies such as several infectious organisms (Coxiella burnett; Epstein-Barr Virus, EBV; cytomegalovirus, CMV; and hepatitis A virus), allopurinol induced hepatitis, Hodgkin's lymphoma, and peripheral T-cell lymphoma.. We retrospectively reviewed 24 patients diagnosed with FRG by bone marrow biopsy at a single institute between 1995 and 2004. We reviewed clinical symptoms and laboratory findings of the patients, classified them by etiology, and compared prognosis of each group.. The most common cause of FRG was acute or chronic EBV infection. Chronic or acute EBV infection was associated with 41.4% of patients (10/24). Of the remaining patients, 33.3% (8/24) were leukemia or lymphoma patients after chemotherapy, 4.2% (1/24) was a patient with hepatic failure, and 20.8% (5/24) were diagnosed as fever of unknown origin. The most common symptom and clinical finding were fever and cytopenia. EBV-associated group comprised chronic active EBV infection, EBV-associated hemophagocytic histiocytosis, acute EBV infection, EBV-associated lymphoproliferative disease, and Langerhans' cell histiocytosis. The EBV-associated group showed a lower survival probability compared with the non-EBV group (P<0.05).. Patients with bone marrow fibrin ring granuloma accompanied by fever require an active workup to find out the cause of infectious agents including EBV infection particularly due to their poor prognosis.

Topics: Adolescent; Adult; Aged; Bone Marrow Diseases; Child; Child, Preschool; Epstein-Barr Virus Infections; Female; Fibrin; Granuloma; Herpesvirus 4, Human; Humans; In Situ Hybridization; Male; Middle Aged; Polymerase Chain Reaction; Prognosis; Q Fever; Retrospective Studies; Survival Rate