Primary immunodeficiencies in the Netherlands: National patient data demonstrate the increased risk of malignancy

•In the Netherlands 745 patients were registered in the national ESID registry.•Prevalence of PID in the Netherlands is 4.0:100,000.•Results show an increased risk for malignancies compared to the general population.•Most common malignancies were lymphomas and skin tumors (other than melanoma).•No malignancies were yet observed related to hematopoietic stem cell transplantation.

PurposeTo analyze the data of the national registry of all Dutch primary immune deficiency (PID) patients, according to the European Society for Immunodeficiencies (ESID) definitions.ResultsIn the Netherlands, 745 patients had been registered between 2009 and 2012. An overall prevalence of 4.0 per 100,000 inhabitants was calculated. The most prevalent PID was 'predominantly antibody disorder (PAD)' (60.4%).In total, 118 transplantations were reported, mostly hematopoietic stem cell transplantations (HSCT). Almost 10% of the PID patients suffered from a malignancy, in particular 'lymphoma' and 'skin cancer'. Compared to the general Dutch population, the relative risk of developing any malignancy was 2.3-fold increased, with a > 10-fold increase for some solid tumors (thymus, endocrine organs) and hematological disease (lymphoma, leukemia), varying per disease category.ConclusionsThe incidence rate and characteristics of PID in the Netherlands are similar to those in other European countries. Compared to the general population, PID patients carry an increased risk to develop a malignancy.