lymphomatoid granulomatosis

Digital cases

 JRC:1608 : Pulmonary lymphomatoid granulomatosis.
 JRC:19434 : Pulmonary lymphomatoid granulomatosis.

Definition: Lymphomatoid granulomatosis is a rare lesional syndrome associated with Epstein-Barr virus EBV-associated B-cell LPD or peripheral T-cell lymphoma.

Lymphomatoid granulomatosis is an angio-destructive lymphoproliferative disorder (LPD) with prominent pulmonary involvement.

Most LYG are an Epstein-Barr virus (EBV)-associated B-cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma) and can be considered as a rare angiocentric and angiodestructive form of EBV-associated B-cell LPD.

Some are associated with peripheral T-cell lymphoma (7573693, 11085664).

Synopsis

 angiocentric immunoproliferative lesion

• angiocentric or angiodestructive lesions with lymphocytic infiltrates (angioinvasion)
• marked angiocentric lymphohistiocytic infiltrate, composed predominantly of CD4-positive T-cells, with angiodestruction and necrosis

 zonal necrosis
 atypical tumoral cells

• associated EBV-associated peripheral B-cell lymphoma
• associated peripheral T-cell lymphoma (11085664)

Localization

 pulmonary lymphomatoid granulomatosis
 cerebral lymphomatoid granulomatosis (20%)
 cutaneous lymphomatoid granulomatosis (11688570)
 digestive lymphomatoid granulomatosis
 renal lymphomatoid granulomatosis
 hepatic lymphomatoid granulomatosis

Predisposition

 secondary immunodeficiencies

• HIV infection and AIDS (11296596, 2703227)
• chemotherapy (11331732, 12571471)
• autologous stem cell transplantation
• immunosuppression
  • immunosuppression for allograft (12802639, 1104468)
  • immunosuppression for auto-immune disease
    • immunosuppression for pemphigus (686725)

• • immunosuppression for dysimmune disease
    • immunosuppression for Crohn disease

 primary immunodeficiencies

• Wiskott-Aldrich syndrome (WAS)
• common variable immunodeficiency (CVID) (12803130)

Associations

 Wegener granulomatosis (12136927)

References

 Guinee DG Jr, Perkins SL, Travis WD, Holden JA, Tripp SR, Koss MN. Proliferation and cellular phenotype in lymphomatoid granulomatosis: implications of a higher proliferation index in B cells. Am J Surg Pathol. 1998 Sep;22(9):1093-100. PMID: 9737242

 Guinee D Jr, Jaffe E, Kingma D, Fishback N, Wallberg K, Krishnan J, Frizzera G, Travis W, Koss M. Pulmonary lymphomatoid granulomatosis. Evidence for a proliferation of Epstein-Barr virus infected B-lymphocytes with a prominent T-cell component and vasculitis. Am J Surg Pathol. 1994 Aug;18(8):753-64. PMID: 8037289

 Myers JL, Kurtin PJ, Katzenstein AL, Tazelaar HD, Colby TV, Strickler JG, Lloyd RV, Isaacson PG. Lymphomatoid granulomatosis. Evidence of immunophenotypic diversity and relationship to Epstein-Barr virus infection. Am J Surg Pathol. 1995 Nov;19(11):1300-12. PMID: 7573693