Abstract
Primary immunodeficiencies (PIDs) are genetically determined irreversible dysfunctions of the immune system. Along with infectious and autoimmune complications, patients with PIDs have high incidence of various malignancies, primarily lymphomas. We analyzed histories of the disease of 27 patients with lymphomas and PIDs, observed at the Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev (Moscow), and compared our results with the data from American Immunodeficiency Cancer Registry (ICR). According to our data, patients with chromosomal breakage syndromes and unspecified PIDs predominate in the group of patients with lymphomas and PIDs. High proportion of patients with Nijmegen breakage syndrome (29%) in our group of patients with lymphomas could be explained by the Slavic mutation in the East European population. PIDs in patients with lymphomas are often diagnosed late - only after the development of lymphoma. Early diagnosis of PIDs and alertness with respect to malignancies in patients with certain PIDs are very important and dictate the need of conduction of studies of malignant lymphoproliferative diseases in these patients.
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