About this Research Topic
The risk of malignancies is higher in patients with primary immunodeficiency (PID) than in the general population. However, the degree of tumor pre-disposition and the underlying mechanisms involved are diverse among various categories of PID. In addition to perturbed tumor immune surveillance in PID and chronic inflammation or infections, the molecular defect per se that causes PID may also facilitate tumorigenesis. This suggests that malignancy in PID may be either a consequence of, or occur in parallel to, the immune defect involved. Furthermore, in some rare syndromes, an observed PID may even be seen as side effect of tumor predisposition, such as in patients harboring mutations in DNA repair genes. In addition, deregulation of epigenetic factors such as, epigenetic changes caused by treatment for PID or in relation to the microbiome are also known to contribute to tumor predisposition in PID.
The disastrous alliance of immunodeficiency with tumor predisposition is both challenging from the basic scientific point of view and from the clinical side, and joint efforts between oncologists and clinical immunologists are needed. The implications are multiple and form the area of the discussion to be addressed herein. In this Research Topic, we welcome the submission of Original Research, Reviews, Mini-Reviews, Case Reports and Perspective articles that address the following questions:
1. Which molecular mechanisms of tumorigenesis are unique in PID and which ones are shared with otherwise healthy individuals?
2. How can we improve the management of patients with PID to prevent malignancy?
3. How does solid cancer or leukemia treatment need to be modified in patients with PID to reduce toxicity and increase the comparably poor cure rate?
4. Are there types of PID that preclude cellular or antibody-mediated immunotherapy of cancer?
5. Which targeted treatment strategies are favorable and unfavorable with respect to the cancer risk of PID patients?
6. Should oncologists be aware of and aim for the diagnosis of pre-existing PID in order to stratify patients and improve their oncological treatment?
7. What may be learned from malignancy in PID to optimize current cancer immune therapy strategies for the general population?
Keywords: Primary immunodeficiency, PID, Cancer predisposition
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