Abstract

Introduction: Primary immunodeficiencies are relatively rare and heterogeneous disorders in which one or more component of immune system have genetic defect. The majority of the patients suffer from frequent infections with different microorganisms according to the type of disorder they have. Some people with primary immunodeficiency are susceptible to malignant diseases. The type of malignancy depends on the type of primary immunodeficiency, age of the patient and the type of virus infection. Many advances have been made in understanding of molecular mechanisms in humeral and cellular immune defects. Furthermore، our knowledge has been increased about the incidence of infections and tumors in primary immunodeficiency and this helps us in early diagnosis of disorders and their complications. Several evidences support the theory that the immune system plays a major role in the occurrence of many malignant diseases. Articles have been searched on Google Scholar، PubMed، Web of Science، and Scopus databases. In this review، the latest and most reliable articles related to the subject that had been published in English language were used.
 Conclusion: Since the patients with primary immunodeficiency are prone to multiple infections, various malignancies can be seen in these people. The recurrence of some malignancies in families, especially blood malignancies may be due to primary immunodeficiency and we should consider evaluation of immune system.

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