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Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Hypergammaglobulinemia overview

Introduction

Overview

Overview

Hypergammaglobulinemia is a primary immunodeficiency disease with an elevated concentration of gamma globulins (immunoglobulins or antibodies).[1][2][3] The term “hypergammaglobulinemia” refers to the position of the excess of proteins after serum protein electrophoresis (found in the gammaglobulin region).[1] The majority of hypergammaglobulinemias are due to an excess of immunoglobulin M (IgM) because this is the default immunoglobulin type prior to class switching.[1] Hypergammaglobulinemias may also result from a deficiency in the other major types of immunoglobulins (IgA, IgE, and IgG).[1][4]

Classification

Classification

  • Hypergammaglobulinemia may be classified into the following:
    • Hyper Ig M associated hypergammaglobulinemias
    • Hyper Ig D syndrome
    • Hyper Ig E syndrome
  • Hyper IgM associated hypergammaglobulinemias are further classified into the following five types:[5][6]
Type Gene involved Mode of inheritance

Type 1

  • Tumor Necrosis Factor Super Family member 5 (TNFSF5) gene encoding CD40L

X-linked recessive

Type 2

  • Activation-Induced Cytidine Deaminase (AICDA) gene

Autosomal recessive

Type 3

  • CD40 gene

Autosomal recessive

Type 4

  • Not yet known

Type 5

  • Gene encoding Uracil-DNA glycosylase

Autosomal recessive

References

References

  1. ↑ 1.0 1.1 1.2 1.3 Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.
  2. ↑ Globulin. Wikipedia. https://en.wikipedia.org/wiki/Globulin. Wikipedia. Accessed on February 24, 2016.
  3. ↑ Primary immunodeficiency. Wikipedia. https://en.wikipedia.org/wiki/Primary_immunodeficiency Accessed on March 2, 2016.
  4. ↑ Immunoproliferative disorder. Wikipedia. Accessed on February 24, 2016.
  5. ↑ Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016
  6. ↑ Immunodeficiency with hyper-IgM, Type 1; HIGM1. http://www.omim.org/entry/308230. Accessed on February 24, 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]