Lymphoma & related disorders
Mature B cell neoplasms
Large B cell lymphomas-special subtypes
Fibrin associated large B cell lymphoma

Editorial Board Member: Elizabeth Courville, M.D.
Deputy Editor-in-Chief: Genevieve M. Crane, M.D., Ph.D.
Jordan M. Hall, M.D.

Topic Completed: 30 March 2021

Minor changes: 30 March 2021

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PubMed Search: Fibrin associated B cell lymphoma

Jordan M. Hall, M.D.
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Cite this page: Hall JM. Fibrin associated large B cell lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomafibrinassoc.html. Accessed April 18th, 2021.
Definition / general
  • Very rare, indolent, non mass forming, Epstein-Barr virus (EBV)+ large B cell lymphoproliferative disorder
  • Noted incidentally on histological examination of tissues excised for some other reason (Am J Surg Pathol 2017;41:299)
  • Neoplastic cells are typically found within fibrinous or amorphous material
Essential features
  • Very rare, indolent, microscopic, EBV+ large B cell lymphoproliferative disorder
  • Localized to a restricted anatomic site
  • Non mass forming, noninvasive with no direct clinical signs or symptoms
  • Noted incidentally on histological examination of tissues excised for other reasons
  • Location of lymphoma cells limited to fibrinous or amorphous eosinophilic material
  • No infiltration or effacement of adjacent normal tissues
  • Excellent prognosis, usually with surgical excision alone
Terminology
  • Fibrin associated (EBV+) large B cell lymphoma (FA DLBCL)
  • Subtype of: diffuse large B cell lymphoma associated with chronic inflammation
ICD coding
  • ICD-10: C83.39 - diffuse large B cell lymphoma, extranodal and solid organ sites
  • ICD-11: 2A81.7 - diffuse large B cell lymphoma associated with chronic inflammation
  • ICD-O: 9680/3 - malignant lymphoma, large B cell, diffuse, NOS
Epidemiology
  • EBV associated
  • Median age 55.5 years
  • M:F = 3:1
  • No ethnic or racial predilection
Sites
Pathophysiology
  • Unknown at this time
Etiology
Clinical features
Diagnosis
  • Requires a high index of suspicion
  • Lymphoma cells incidentally noted only on microscopic examination of tissues
  • Exclude systemic or invasive disease and primary or iatrogenic immunosuppression
  • Localized to a restricted anatomic site
Radiology description
  • No evidence of lymphoma on imaging (if performed)
Prognostic factors
Case reports
Treatment
  • Surgical excision alone is usually adequate
Gross description
  • No gross tumor mass or evidence of lymphoma
Microscopic (histologic) description
  • Small foci of lymphoma cells in fibrinous or amorphous eosinophilic material
    • Aggregates / clusters
    • Ribbons
    • Single cells
  • Neoplastic cells are large and atypical
    • Irregular nuclear contours
    • Coarse chromatin
    • Distinct nucleoli
    • Amphophilic cytoplasm
    • May be plasmacytoid or bizarre appearing
    • Mitotic figures and apoptotic bodies present
  • Lack of lymphomatous infiltration or effacement of adjacent normal tissues
  • Usually lacks prominent inflammatory background in vicinity of lymphoma cells
Microscopic (histologic) images

Contributed by Jordan M. Hall, M.D.

Knee synovium

Knee joint space fibrin

PAX5

CD30

Ki67


EBER ISH

Positive stains
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Soft tissue, right subpatellar space, excision:
    • Fibrin associated diffuse large B cell lymphoma (see comment)
    • Comment: Histopathologic evaluation reveals fragments of synovium with patchy chronic inflammation and abundant eosinophilic fibrinous material. Within and limited to the fibrin, there are scattered small collections of large atypical lymphoid cells noted. Immunohistochemistry shows these cells are positive for CD20, PAX5, CD30 and CD43, express an activated B cell immunophenotype (CD10 negative, BCL6 negative, MUM1 positive) and show a high proliferative rate (> 90% Ki67 positivity). These cells are also positive for Epstein-Barr virus encoded small RNA (EBER) by chromogenic in situ hybridization. Negative stains include CD3, CD15, CD68, CD163, CD138, ALK1 and SOX11. Impression: The histopathologic findings and available clinical history of arthroplasty without clinical suspicion for neoplasia support a diagnosis of FA DLBCL. This is a clinically indolent lymphoproliferative disorder that is confined to a restricted anatomic space, typically an incidental finding on histopathologic examination only and usually adequately treated with surgical excision alone. That being said, correlation with the complete clinical and radiologic workup is recommended to exclude the possibility of more aggressive systemic disease.
Differential diagnosis
Board review style question #1

    Which of the following is detected in the tumor cells of fibrin associated diffuse large B cell lymphoma?

  1. HHV8
  2. EBV
  3. KSHV
  4. HIV
  5. HSV
Board review style answer #1
Board review style question #2

    Fibrin associated diffuse large B cell lymphoma is currently included in the 2017 revised WHO classification as a subtype of which entity?

  1. EBV positive diffuse large B cell lymphoma, NOS
  2. High grade B cell lymphoma
  3. Anaplastic large cell lymphoma
  4. Diffuse large B cell lymphoma associated with chronic inflammation
  5. Primary effusion lymphoma
Board review style answer #2
D. Diffuse large B cell lymphoma associated with chronic inflammation

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Reference: Fibrin associated large B cell lymphoma
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