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Hypergammaglobulinemia classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]

Overview

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:[1][2]
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

  1. Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016
  2. Immunodeficiency with hyper-IgM, Type 1; HIGM1. http://www.omim.org/entry/308230. Accessed on February 24, 2016

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