Extranodal NK-T-cell lymphoma, nasal type in granulomatosis with polyangiitis. A case report

Granulomatosis with polyangiitis (GP) is a systemic necrotizing vasculitis with multi-organ involvement that primarily affects the respiratory tract and the kidneys. Fever in these patients is an indicator of activity; however, if it arises in isolation, the physician should exclude other causes. Ma...

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Published inRevista medica del Instituto Mexicano del Seguro Social Vol. 55; no. 3; p. 394
Main Authors Mendoza-Álvarez, Sergio Alberto, Rodríguez-Dávila, Fátima Margarita, Moranchel-García, Leslie, Soto, Virginia, Quisped, Natalia
Format Journal Article
LanguageSpanish
Published Mexico 01.05.2017
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Summary:Granulomatosis with polyangiitis (GP) is a systemic necrotizing vasculitis with multi-organ involvement that primarily affects the respiratory tract and the kidneys. Fever in these patients is an indicator of activity; however, if it arises in isolation, the physician should exclude other causes. Male patient admitted due to an unexplained fever and weight loss; it was diagnosed GP by a history of pauci-immune glomerulonephritis, fixed pulmonary nodules and chronic sinusitis of four years of evolution; however, the fever persisted despite treatment and in the absence of infection. It was performed an oropharynx lesion biopsy and the diagnosis was extranodal NK-T-cell lymphoma, nasal type, and positive for CD56 and granzyme. Extranodal NK-T-cell lymphoma, nasal type is a rare entity, of poor prognosis, that should be considered as a diagnosis in patients with GP unresponsive to steroid. That is the reason why biopsy of the lesion and immunohistochemistry are required.
ISSN:2448-5667