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Immunodeficiency Disorders

Introduction

Immunodeficiency diseases are a group of disorders that increase susceptibility to infection, malignancy, and autoimmunity. Deficiencies of the immune system may be congenital or acquired. They may be secondary to a developmental anomaly or an enzymatic defect or be of unknown etiology. Recently it has become clear that immune responses are comprised of a large number of discrete steps, and there may be a breakdown at any point in this process. It is likely that hundreds of different lesions lead to immunodeficiency diseases. It is important to remember that it was from studies of patients who had abnormalities of the immune system that many of the functions and components of the normal immune system have been elucidated. There are two broad categories of immunodeficiencies: those that are primary and those that are secondary to another disorder.

Classification and Incidence

The immune system has four major components: 1) antibody-mediated (B cell) immunity, 2) cell-mediated (T cell) immunity, 3) phagocytosis (polymorphonuclear and mononuclear cells), and 4) the complement system. This division provides a practical way to classify the immunodeficiencies (Table 1). Common secondary immunodeficiencies are listed in Table 2. A more complete classification of primary immunodeficiencies is that of the 1986 WHO Scientific group (see Suggested Reading).

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