Sociedad Argentina de Hematología

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Revista Argentina de Hematología


Volumen:    21    # Number : 1

Publication Date :    Enero - Abril    Year:    2017


IgG4-related disease: a diagnostic challenge

Authors: Agazzoni M, Barrenechea M, Bolaños S, Brambilla D, Egozcue C, Kalmus M, Lozano L, Peña C, Rojas E

Abstract: IgG4-related disease (IgG4-RD) it is a clinicopathological entity recently described, with a broad spectrum of clinical manifestations that share a common histopathology. Arriving to diagnosis is difficult, requiring clinical, laboratory and histopathology complementary findings. Usually a good response is observed after treatment with steroids; however there are a few case reports of successful therapy with immunosupressors and B cell depletory drugs. This case report allows depicting the diagnosis challenge that this entitiy often entails.

Key words: IgG4, IgG4-related disease, plasma cells, fibrosis.

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