Primary disorders (PID) are inborn errors of the immune system. (1,2). Developed countries have registries to estimate the prevalence, incidence, and patterns of PIDs. Costa Rica is a country with a population of 5 million where pediatric PIDs are followed in one center that is part of the Latin American Society for immunodeficiencies (LASID)(1,3). Among the latin-american countries Costa Rica represents the 6% of all reported cases of PIDs (1).
The epidemiology of major PIDs diagnosis and the frequency of malignancy at the National Children´s Hospital (HNN), San José Costa Rica is described in period of ten years.
We performed a preliminary retrospective analysis of the patients that attended our clinic in the last 10 years and describe the malignancy presentation.
Table 1: Number of cases of the most common PIDs and number of cases with malignancy
| PID | No. Cases | Malignancy |
| IPEX | 1 | 0 |
| PI3KCD | 1 | 0 |
| DOCK8 | 2 | 1 |
| PIK3R1 | 2 | 1 |
| IKARUS | 2 | 0 |
| C1-INH | 2 | 0 |
| FHL-4 | 3 | 1 |
| SIgAD | 4 | 0 |
| WAS | 4 | 0 |
| CD40LD | 4 | 0 |
| CGD | 12 | 0 |
| XLA | 17 | 0 |
| SCID | 19 | 0 |
| AT | 97 | 8 |
| TOTAL | 160 | 11 |
AT is the most common PID in Costa Rica and it represents 60% of the patients registered so far. Malignancy presented predominately in AT patients as expected. It was distributed as 1 gastric adenocarcinoma, 2 nasopharyngeal NHL, 1 T-cell lymphoma, 1 ovarian cancer, 1 hepatoblastoma. 2 AT patients had two malignancies (non Hodgkin and Hodgkin lymphoma).
Costa Rica has a high prevalence of AT.