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.

Last author update: 30 March 2021
Last staff update: 28 July 2023

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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 May 19th, 2024.
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., Hyunkyu Shin, M.D., Dr. Christian Schürch, M.D., Ph.D., Falko Fend, M.D. and Claudia Wickenhauser, M.D., Ph.D. (Case #529)

Knee synovium

Knee joint space fibrin

PAX5

CD30

Ki67


EBER ISH

H&E staining H&E staining H&E staining H&E staining H&E staining

H&E staining


H&E staining H&E staining H&E staining

H&E staining

CD138

CD138

MUM1

MUM1

CD3

CD3


CD20

CD20

CD79a

CD79a

ALK

ALK

MYC

MYC

HHV8

HHV8

Kappa

Kappa


Lambda

Lambda

Cyclin D1

Cyclin D1

CD56

CD56

EBER

EBER

BCMA

BCMA

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. EBV
  2. HHV8
  3. HIV
  4. HSV
  5. KSHV
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. Anaplastic large cell lymphoma
  2. Diffuse large B cell lymphoma associated with chronic inflammation
  3. EBV positive diffuse large B cell lymphoma, NOS
  4. High grade B cell lymphoma
  5. Primary effusion lymphoma
Board review style answer #2
B. Diffuse large B cell lymphoma associated with chronic inflammation

Comment Here

Reference: Fibrin associated large B cell lymphoma
Board review style question #3
    Which of the following is a true feature of fibrin associated large B cell lymphoma (FA-LBCL)?

  1. Distinguishing this tumor from chronic inflammation associated (CI-)LBCL has no clinical importance because both are inflammation associated indolent neoplasms
  2. The neoplastic cells are derived from germinal center B cells
  3. This neoplasm is strongly associated with EBV infection with type III latency
  4. This tumor is a highly aggressive lymphoid neoplasm due to its association with acute fibrous inflammation
  5. With discohesive neoplastic cells, this tumor readily infiltrates pre-existing anatomic structure
Board review style answer #3
C. This neoplasm is strongly associated with EBV infection with type III latency. Except for rare cases as presented here, FA-LBCL is consistently associated with EBV infection showing type III latency with LMP1 and EBNA2 positivity. Answer A is incorrect because CI-LBCL exhibits a more aggressive clinical course than FA-LBCL with similar morphology, so these two entities should be clearly distinguished. Answer B is incorrect because the neoplastic cells of FA-LBCL show an activated (nongerminal center) B cell phenotype (CD10-, BCL6+/-, MUM1+). Answer D is incorrect because FA-LBCL is an indolent lymphoid neoplasm, so it is often discovered incidentally during careful examination. Answer E is incorrect because this tumor is mostly confined to cystic or pseudocystic spaces without infiltration of pre-existing anatomic structures or mass formation.

Comment Here

Reference: Fibrin associated large B cell lymphoma
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