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pulmonary lymphomatoid granulomatosis

Tuesday 16 November 2004

Digital case

- 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. 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

- primary immunodeficiencies

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

Portfolio

  • Pulmonary nodule in lymphomatoid granulomatosis
  • Pulmonary nodule in lymphomatoid granulomatosis