Rosay–Dorfman – like lymphadenopathy in a patient with Wiskott–Aldrich syndrome: diagnostic difficulties

Abstract

Wiskott–Aldrich Syndrome (WAS) is a primary immunodeficiency (PID), characterized by varying severity of typical symptoms: thrombocytopenia, infections, immune dysregulation and cancer predisposition. Therefore any lymphoproliferative complications in WAS patients require lymph node biopsy. However, the interpretation of the histological picture is often complicated and requires the knowledge of the lymph node pathomorphology in patients with immunodeficiency. This article describes a rare combination of late diagnosis of WAS complicated by lymphadenopathy, which clinically and morphologically resembles Rosai–Dorfman disease (RDD). Parents gave their consent to use information about the child, including the fotos, in the article.