Lymphomatoid granulomatosis is a rare lesional syndrome associated with Epstein-Barr virus EBV-associated B-cell LPD. It 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. However, 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
 tumor nodules: mixture of atypical large CD20+ lymphocytes, with vesicular nuclei and prominent nucleoli and with a background of small and intermediate-size T-lymphocytes, histiocytes, and plasma cells.
 EBV1-encoded RNA present in CD20+ large, atypical lymphocytes

Predisposition

 secondary immunodeficiencies

• HIV infection (#11296596#)
• chemotherapy (#11331732#, #12571471#)
• autologous stem cell transplantation
• organ allograft (#12802639#)

 primary immunodeficiencies

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

Subtypes

 grade 1 lymphomatoid granulomatosis
 grade 2 lymphomatoid granulomatosis
 grade 3 lymphomatoid granulomatosis

Differential diagnosis

 Wegener granulomatosis
 varicella-zoster-related pneumonia
 bronchocentric granulomatosis
 Hodgkin lymphoma
 peripheral T-cell lymphoma (PTCLs)
 natural killer cell lymphoma (NKCL)

Other localization

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

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#