Lymphomatoid granulomatosis: A practical review for pathologists dealing with this rare pulmonary lymphoproliferative process

E. Tagliavini, G. Rossi, R. Valli, M. Zanelli, A. Cadioli, M. C. Mengoli, A. Bisagni, A. Cavazza, G. Gardini

Research output: Contribution to journalArticlepeer-review

Abstract

Lymphomatoid granulomatosis (LYG) is a rare B-cell lymphoproliferative disorder predominantly involving the lungs, but poorly-recognized among clinicians and pathologists. It is an Epstein-Barr virus (EBV)-driven disease mimicking several other diseases on clinical and radiological grounds, generally showing multiple, bilateral nodular, ill-defined infiltrates of the lungs tending to coalescence and/or cavitation. LYG often affects middleaged males with an underlying immunodeficiency and commonly involves skin and central nervous system during disease progression. Diagnosis requires a generous biopsy and careful histologic examination with immunohistochemical staining and molecular demonstration of EBV genome in large atypical B-cells. LYG is graded as I to III based on the number of large EBV-positive B-cells; grades II/III are now considered as a peculiar variant of T-cell rich diffuse large B-cell lymphoma. In this brief review, clinical, radiologic and pathologic features of LYG will be analyzed with focus on differential diagnosis, the most appropriate treatment and prognosis.

Original languageEnglish
Pages (from-to)111-116
Number of pages6
JournalPathologica
Volume105
Issue number4
Publication statusPublished - Aug 2013

Keywords

  • EBV
  • Immunodeficiency
  • Immunohistochemistry
  • Lung
  • Lymphoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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