Fibrin-associated diffuse large B-cell lymphoma
Fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is an extremely rare form of the diffuse large B-cell lymphomas (DLBCL). DLBCL are lymphomas in which a particular type of lymphocyte, the B-cell, proliferates excessively, invades multiple tissues, and often causes life-threatening tissue damage. DLBCL have various forms as exemplified by one of its subtypes, diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI). DLBCL-CI is an aggressive malignancy that develops in sites of chronic inflammation that are walled off from the immune system. In this protected environment, the B-cells proliferate excessively, acquire malignant gene changes, form tumor masses, and often spread outside of the protected environment. In 2016, the World Health Organization provisionally classified FA-DLBCL as a DLBCL-CI. Similar to DLBCL-CI, FA-DLBCL involves the proliferation of EBV-infected large B-cells in restricted anatomical spaces that afford protection from an individual's immune system. However, FA-DLBCL differs from DLBCL-CI in many other ways, including, most importantly, its comparatively benign nature. Some researchers have suggested that this disease should be regarded as a non-malignant or pre-malignant lymphoproliferative disorder rather than a malignant DLBCL-CI.

FA-DLBCL is an Epstein–Barr virus-associated lymphoproliferative disease (EBV+ LPD), i.e. disease in which lymphocytes infected with the Epstein-Barr virus (EBV) proliferate excessively in one or more tissues. EBV infects ~95% of the world's population to cause no symptoms, minor non-specific symptoms, or infectious mononucleosis. The virus then enters a latency phase in which the infected individual becomes a lifetime asymptomatic carrier of the virus. Some weeks, months, years, or decades thereafter, a very small fraction of these carriers develop any one of various EBV-associated benign or malignant diseases. In FA-DLBCL as well as DLBCL-CI, EBV infects B-cells to promote their proliferation and thereby the development of either disease.

FA-DLBCL most commonly develops within immunologically sequestered sites such as body cavities (e.g. pseudocysts) and foreign bodies (e.g. artificial heart valves) where fibrin, a breakdown product of the blood clotting factor, fibrinogen, has deposited. It is almost always discovered as an incidental finding in specimens taken from these sites when they are examined for reasons not directly related to the FA-DLBCL infiltrates. As reviewed in a publication of 2019, the disorder has been diagnoses in 47 individuals who are predominantly elderly males; it is almost uniformly amenable to various treatments and takes a benign course. However, the disease, when occurring within vascular or cardiac sites, does have a risk of being complicated by the development of embolisms due to the dislodgement of blood clots that travel through the vascular system to cause, e.g. strokes.
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diffuse large B-cell lymphoma
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lymphomas
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diffuse large B-cell lymphoma associated with chronic inflammation
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vascular system
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Fibrin-associated diffuse large B-cell lymphoma
Concept
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