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

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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.

About the authors

O. A. Shvets

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation

Author for correspondence.
Email: shv1808@rambler.ru
ORCID iD: 0000-0001-5347-7150

MD, pediatrician allergologist-immunologist, 

117997, Moscow, Samory Mashela st., 1

Russian Federation

D. S. Abramov

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation

ORCID iD: 0000-0003-3664-2876
Moscow Russian Federation

A. L. Khoreva

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation

ORCID iD: 0000-0002-7684-9188
Moscow Russian Federation

D. E. Pershin

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation

ORCID iD: 0000-0002-6148-7209
Moscow Russian Federation

A. M. Kieva

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation

ORCID iD: 0000-0003-2467-2840
Moscow Russian Federation

A. V. Phonkin

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation

ORCID iD: 0000-0002-2057-2036
Moscow Russian Federation

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Copyright (c) 2020 Shvets O.A., Abramov D.S., Khoreva A.L., Pershin D.E., Kieva A.M., Phonkin A.V.

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