Hyper-IgM Immunodeficiency Syndrome

A rare inherited immunodeficiency syndrome characterized by normal or elevated serum IMMUNOGLOBULIN M levels with absence of IMMUNOGLOBULIN G; IMMUNOGLOBULIN A; and IMMUNOGLOBULIN E. It results in a profound susceptibility to BACTERIAL INFECTIONS and an increased susceptibility to OPPORTUNISTIC INFECTIONS. Several subtypes of hyper-IgM immunodeficiency syndrome exist depending upon the location of genetic mutation.

Also Known As:

Hyper-IgM Syndrome; HIGM2 Syndrome; HIGM3 Syndrome; HIGM5 Syndrome; Hyper-IgM Immunodeficiency Syndrome Type 2; Hyper-IgM Immunodeficiency Syndrome Type 3; Hyper-IgM Immunodeficiency Syndrome Type 5; Hyper-IgM Syndrome 2; Hyper-IgM Syndrome 3; Hyper-IgM Syndrome 5; Immunodeficiency with Hyper-IgM Syndrome; Immunodeficiency with Hyper-IgM, Type 2; Immunodeficiency with Hyper-IgM, Type 3; Immunodeficiency with Hyper-IgM, Type 5; HIGM2 Syndromes; HIGM3 Syndromes; HIGM5 Syndromes; Hyper IgM Immunodeficiency Syndrome; Hyper IgM Immunodeficiency Syndrome Type 2; Hyper IgM Immunodeficiency Syndrome Type 3; Hyper IgM Immunodeficiency Syndrome Type 5; Hyper IgM Immunodeficiency Syndrome, Type 2; Hyper IgM Immunodeficiency Syndrome, Type 3; Hyper IgM Immunodeficiency Syndrome, Type 5; Hyper IgM Syndrome; Hyper IgM Syndrome 2; Hyper IgM Syndrome 3; Hyper IgM Syndrome 5; Hyper-IgM Immunodeficiency Syndromes; Hyper-IgM Syndrome 5s; Hyper-IgM Syndromes; Immunodeficiency Syndrome, Hyper-IgM; Immunodeficiency Syndromes, Hyper-IgM; Immunodeficiency with Hyper IgM Syndrome; Immunodeficiency with Hyper IgM, Type 2; Immunodeficiency with Hyper IgM, Type 3; Immunodeficiency with Hyper IgM, Type 5; Syndrome, Hyper-IgM Immunodeficiency; Syndromes, Hyper-IgM Immunodeficiency; Hyper-IgM Immunodeficiency Syndrome, Type 2; Hyper-IgM Immunodeficiency Syndrome, Type 3; Hyper-IgM Immunodeficiency Syndrome, Type 5

Hemic and Lymphatic Diseases: 6
- Hematologic Diseases: 3366
  - Blood Protein Disorders: 17
    - Dysgammaglobulinemia: 77
      - Hyper-IgM Immunodeficiency Syndrome: 187
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Primary Immunodeficiency Diseases: 1189
    - Hyper-IgM Immunodeficiency Syndrome: 187
  - Hyper-IgM Immunodeficiency Syndrome: 187
Immune System Diseases: 4321
- Immunologic Deficiency Syndromes: 91
  - Primary Immunodeficiency Diseases: 1189
    - Hyper-IgM Immunodeficiency Syndrome: 187
  - Dysgammaglobulinemia: 77
    - Hyper-IgM Immunodeficiency Syndrome: 187

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Disease

Drug/Important Bio-Agent (IBA)