Immunodeficiency Disorders Due to Antibody Deficiency (B‐Lymphocyte Disorders)

Abstract

The majority of patients with primary immunodeficiency have a defect in their ability to make sufficient quantities of specific antibodies. This lack of specific antibodies is due to the lack of B lymphocytes, defects in B‐lymphocyte function or inability of B lymphocytes to interact with T lymphocytes. Patients born with only antibody deficiency usually appear normal at birth owing to maternal antibodies that crossed the placenta. They usually start developing more infections at around 6 months of age when maternal antibodies have been depleted, and they cannot make their own antibodies. Treatment may include prophylactic antibodies until diagnosis is confirmed or infusion of immunoglobulin, either by intravenous route or subcutaneously. When patients have antibody deficiency plus defects in T lymphocytes, the disease is usually more severe and will be further discussed in other articles.

Key Concepts

  • The most severe form of antibody deficiency, Bruton's, requires regular infusions of intravenous immunoglobulin starting in infancy.
  • Common variable immunodeficiency (CVID) is often associated with autoimmune disease as well as malignancies.
  • Less severe forms of B‐cell deficiency may be managed with prophylactic antibiotics.
  • Severe combined immunodeficiency (SCID) requires stem cell transplantation for treatment.
  • Early diagnosis is essential in preventing long‐term disability or early death.

Keywords: antibody deficiency; B lymphocytes; Bruton agammaglobulinaemia; immunoglobulins; cytokines

References

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Further Reading

Ochs HD , Smith CIE and Puck JM (eds) (1999) Primary Immunodeficiency Diseases. New York: Oxford University Press.

Rich RR (ed) (1995) Clinical Immunology: Principles and Practice. Portland, Oregon: Mosby.

Rosen FS , Cooper MD and Wedgwood RJP (1995) The primary immunodeficiencies. New England Journal of Medicine 333: 431–440.

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How to Cite close
Roberts, Robert L(Mar 2016) Immunodeficiency Disorders Due to Antibody Deficiency (B‐Lymphocyte Disorders). In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0002166.pub3]