Increased risk of hematologic malignancies in primary immunodeficiency disorders: opportunities for immunotherapy.

Citation data:

Clinical immunology (Orlando, Fla.), ISSN: 1521-7035, Vol: 190, Page: 22-31

Publication Year:
2018
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PMID:
29499421
DOI:
10.1016/j.clim.2018.02.007
Author(s):
Verhoeven, Dorit; Stoppelenburg, Arie Jan; Meyer-Wentrup, Friederike; Boes, Marianne
Publisher(s):
Elsevier BV
Tags:
Medicine; Immunology and Microbiology
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review description
Primary immunodeficiency disorders (PIDs) convey increased susceptibility to infections and sometimes to malignancies, particularly lymphomas. Such cancer development can be contributed by immune impairments resulting in weakened immunological surveillance against (pre)malignant cells and oncogenic viruses. Molecular defects in PID-patients are therefore being clarified, identifying new targets for innovative immunotherapy. Particularly pediatric cancers are being scrutinized, where over one-third of cancer-related deaths are accounted for by leukemia and lymphomas. Here we review how immunopathogenic mechanisms of several PIDs might associate with lymphoma development. We furthermore delineate existing immunotherapy strategies in adults for potential therapeutic application in childhood leukemia and lymphomas.