Advances in the Diagnosis and Treatment of Primary Immunodeficiency Diseases

Abstract

Over the 20-year span of my investigation of patients with heritable disorders of immunity on Duke University's (Durham, NC) Rankin Clinical Research Unit, remarkable progress has been made worldwide in defining the cellular basis of such defects and in improving treatment for them. Considering the vast literature on this subject, it is often difficult to realize that the first human immunodeficiency disease was discovered just 33 years ago.¹ Since then information regarding such defects has accrued at an enormous rate. The World Health Organization appreciated this in the late 1970s and created a committee whose charge was to develop a classification of the then-recognized syndromes. In the first such classification, the authors attempted to separate the conditions according to postulated cellular levels at which the defects occurred and on the basis of consistent patterns of inheritance or clinical manifestations. At the time of the first classification, however, none of