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Primary immunodeficiencies and their associated risk of malignancies in children: an overview

10$
ARTICLE DOWNLOAD

Primary immunodeficiencies and their associated risk of malignancies in children: an overview

10$

Samuele Renzi, Karin Petra Sabine Langenberg-Ververgaert, Nicolas Waespe, Salah Ali, Jack Bartram, Orli Michaeli, Julia Upton & Michaela Cada 

bstract

Primary immunodeficiency disorders represent a heterogeneous spectrum of diseases, predisposing to recurrent infections, allergy, and autoimmunity. While an association between primary immunodeficiency disorders and increased risk of cancer has been suggested since the 1970s, renewed attention has been given to this topic in the last decade, largely in light of the availability of large registries as well as advances in next generation sequencing. In this narrative review, we will give an insight of the primary immunodeficiencies that are commonly responsible for the greater number of cancers in the primary immunodeficiency disorders population. We will describe clinical presentations, underlying genetic lesions (if known), molecular mechanisms for carcinogenesis, as well as some management considerations. We will also comment on the future directions and challenges related to this topic.

Conclusion: The awareness of the association between several primary immunodeficiencies and cancer is crucial to provide the best care for these patients.

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Year 2020
Language English
Format PDF
DOI 10.1007/s00431-020-03619-2