Objective: Inventory of patients with immunodeficiency in the Netherlands.
Design: Retrospective over the period 1970-1983.
Setting: Dutch Working Group on Immunodeficiency, University Children's Hospital, 'Het Wilhelmina Kinderziekenhuis', Utrecht.
Method: A questionnaire with specific information on immunodeficient patients was completed by clinical members of the Working Group i.e. paediatricians and internists. Diagnosis of the immunodeficiency was according to WHO recommendations.
Results: The data of 336 patients could be included and analysed. Defects in humoral immunity were the most common (62.9%), followed by combined defects (18.75%), T cell defects (7.4%), phagocytic disorders (6.8%) and complement component deficiencies (2.7%). Sixty-two patients (15.5%) died, notably those with combined T and B cell defects, selective T cell defects and those with immunodeficiency based on DNA repair defects. Autoimmunity was relatively frequent in immunodeficiency (7.7%) as was cancer (2.7%).
Conclusion: Results were similar to those of other European countries.