Abstract Background We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network ...(USIDNET) registry compared with age-adjusted cancer incidence in the Surveillance, Epidemiology and End Results Program (SEER) database. We hypothesized that subjects with PIDD would have an increased incidence of cancer due to impaired immune function. Methods Overall and site-specific cancer incidence rates were evaluated in subjects with PIDD (n = 3,658) enrolled in the USIDNET registry from 2003-2015, and compared with age-adjusted incidence rates in the SEER database. Results We observed a 1.42-fold excess relative risk of cancer in subjects with PIDD compared to the age-adjusted SEER population (p<0.001). Men with PIDD had a 1.91-fold excess relative risk of cancer compared to the age-adjusted male population (p<0.001), while women with PIDD had similar overall cancer rates compared to the age-adjusted female population. Of the four most common malignancies in men and women in SEER (lung, colon, breast, and prostate cancers), we found no significant increase in these diagnoses in subjects with PIDD. Significant increases in lymphoma in both men (10-fold increase, p<0.001) and women (8.34-fold increase, p<0.001) with PIDD were observed. Conclusions Excess incidence of cancer occurred in subjects with PIDD. An excess of lymphoma in specific PIDD populations principally drove this increased incidence, while no increased risk of the most common solid tumor malignancies was observed. These data point to a restricted role of the immune system in protecting from specific cancers.
A major diagnostic intervention in the consideration of many patients suspected to have primary immunodeficiency diseases (PIDDs) is the application and interpretation of vaccination. Specifically, ...the antibody response to antigenic challenge with vaccines can provide substantive insight into the status of human immune function. There are numerous vaccines that are commonly used in healthy individuals, as well as others that are available for specialized applications. Both can potentially be used to facilitate consideration of PIDD. However, the application of vaccines and interpretation of antibody responses in this context are complex. These rely on consideration of numerous existing specific studies, interpolation of data from healthy populations, current diagnostic guidelines, and expert subspecialist practice. This document represents an attempt of a working group of the American Academy of Allergy, Asthma & Immunology to provide further guidance and synthesis in this use of vaccination for diagnostic purposes in consideration of PIDD, as well as to identify key areas for further research.
...Skevaki et al13 review immune biomarkers in the spectrum of childhood noncommunicable diseases with an emphasis on allergies, autoimmunity, and immune regulation.
More importantly, Donald established JACI as the undisputed number one allergy and clinical immunology journal worldwide.1 We are also personally grateful to Donald for his mentorship, guidance, and ...patience during the transition period, ensuring a seamless crossover. In doing so, we reviewed the report of the Future of Allergy task force, as well as the Needs Assessment surveys conducted by the AAAAI.2 It is clear that the scope of our specialty will be radically changed over the next 5 to 10 years. ...we will be proactive in expanding space allotted to original and review articles discussing emerging topics, therapeutic trends, and policies.
Background The interpretation of an adequate response to the unconjugated 23-valent pneumococcal vaccine for serotypes having high preimmunization titers remains challenging. Objectives We sought to ...determine whether high preimmunization titers preclude a 4-fold or greater response to vaccination. Moreover, we sought to determine the effect of the following covariates on this response: absolute preimmunization titer value, age, sex, serum IgG level, and serum IgG subclasses. Methods We conducted a retrospective analysis of patients who were seen in our immune disorders clinic between 2001 and 2007 who had received the unconjugated 23-valent pneumococcal vaccine. Logistic regression was used to estimate the effect of different covariates, including preimmunization titer values, age, sex, IgG levels, and IgG subclass values, on the odds of a 4-fold or greater antibody response. Results Per serotype, 10% to 40% of subjects with a high preimmunization titer attained at least a 4-fold response to vaccination. However, the odds of a 4-fold or greater response were found to decrease as a function of the absolute preimmunization titer value with an absolute value for each serotype beyond which the odds ratio approached zero. Conclusion High pneumococcal preimmunization titers do not necessarily preclude a 4-fold or greater response to vaccination. However, there appear to be serotype-specific preimmunization titer values, ranging from 4.4 to 10.3 μg/mL, above which a 4-fold or greater response would not be expected. This response does not seem to be significantly affected by age, sex, IgG level, or IgG subclass value.
Rationale While the molecular etiology of primary HLH (pHLH) is well characterized, secondary HLH (sHLH) is less defined. sHLH is a heterogeneous entity whose severity and presentation vary depending ...on the underlying disease. ...the HLH 2004 therapeutic protocol (developed for pHLH) may not be the best approach for all sHLH patients.
The Editors' Choice Akdis, Cezmi A., MD; Ballas, Zuhair K., MD
Journal of allergy and clinical immunology,
2017, Letnik:
139, Številka:
4
Journal Article
The Editors' Choice Akdis, Cezmi A., MD; Ballas, Zuhair K., MD
Journal of allergy and clinical immunology,
2017, Letnik:
139, Številka:
3
Journal Article
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