Immunodeficiency Disorders - Introduction

Introduction
The primary immunologic deficiency diseases include congenital and acquired disorders of humoral immunity (B cell function) or cell-mediated immunity (T cell function). Most of these diseases are rare, and since they are genetically determined, are seen primarily in children. Several immunodeficiency disorders affect adults, and are discussed below. The WHO classification of immunodeficiency disorders more often affecting adults is set forth in the accompanying box.

WHO CLASSIFICATION

  • Primary Immunodeficiency Disorders: Selective IgA deficiency. Common variable immunodeficiency. X-linked agammaglobulinemia. Immunodeficiency with normal serum globulins or hyperimmunoglobulinemia. Immunodeficiency with thymoma.
  • Secondary Immunodeficiency Disorders (for example, AIDS)

Preferences
Bonilla FA et al: Primary immunodeficiency diseases. J Allergy Clin Immunol 2003;111:S571.

Buckley RH: Advances in immunology: primary immunodeficiency diseases due to defects in lymphocytes. N Engl J Med 2000;343:1313.

Fischer A: Human primary immunodeficiency diseases: a perspective. Nat Immunol 2004;5:23.

 

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Revision date: June 20, 2011
Last revised: by Janet A. Staessen, MD, PhD