Background and Aims

Background: Underlying oncogenic mechanisms in PID are multiple and not completely defined, thus, establishing the prevalence of cancer in each particular group of immune disorders is relevant. According to epidemiological data in Argentina, cancer incidence rate in the general pediatric population is 129.9 per million.

Aims: To document cancer incidence in our cohort of PID patients (pts) and PID type involved.

Methods

Review of clinical charts of our Hospital’s PID registry from March/1989 to March/2018 screening for neoplasia occurrence (follow-up:1-18years).

Results

38 from 956 PID pts, (607 males/349 females) developed malignancy(4%). Mean age:7.8 (1-17)years.

Cancer cases distribution according to 2017-IUIS-Classification was:

Immunodeficiencies affecting cellular/humoral immunity: n:2/78(2.6%): 2 Hodgkin Lymphoma(HL).

CID with associated or syndromic features: 22/473pts (4.7%): 15 Non-HL, 2 HL, 3 Leukemia(AL) and 3 Solid tumors. Considering only Ataxia-Telangiectasia, 12/65 developed cancer. No Di George Syndrome patient (n:331) developed neoplasia.

Predominantly Antibody Deficiencies: 11/194pts (4.6%): 4NHL, 3HL, 3 Solid tumors and 1 AL.

Diseases of Immune Dysregulation: 3/82pts (3.7%) 2 NHL, 1AL.

Congenital Phagocyte Defects: 2/84pts (2.4%), although therapy-related carcinomas.

Conclusions

This is the first pediatric registry of malignancy in PID in our country, involving in addition a significant number of cases. The Incidence rate disclosed 1284 cases/million, well above that of the general Argentinian pediatric population. The CID associated to Syndromic features group showed the highest prevalence of cancer. NHL (53%) was the most prevalent neoplasm overall. Documentation and better understanding of oncogenic mechanisms involved will help prevent in some degree the occurrence of neoplasia in PID patients.