Lymphomatoid granulomatosis: a practical review for pathologists dealing with this rare pulmonary lymphoproliferative process.
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 middle-aged 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.
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera Arcispedale Santa Maria Nuova/Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Reggio Emilia, Italy.
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Tagliavini
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera Arcispedale Santa Maria Nuova/Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Reggio Emilia, Italy.
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Rossi
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera Arcispedale Santa Maria Nuova/Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Reggio Emilia, Italy.
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Valli
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera Arcispedale Santa Maria Nuova/Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Reggio Emilia, Italy.
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Zanelli
Struttura Complessa di Anatomia Patologica, Azienda Ospedaliera-Universitaria Policlinico di Modena, Modena, Italy.
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Cadioli
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera Arcispedale Santa Maria Nuova/Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Reggio Emilia, Italy.
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Mengoli
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera Arcispedale Santa Maria Nuova/Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Reggio Emilia, Italy.
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Bisagni
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera Arcispedale Santa Maria Nuova/Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Reggio Emilia, Italy.
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Cavazza
Unità Operativa di Anatomia Patologica, Azienda Ospedaliera Arcispedale Santa Maria Nuova/Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Reggio Emilia, Italy.
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Gardini
D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology*; D008230:Lymphomatoid Granulomatosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D008297:Male; D011379:Prognosis; D011859:Radiography