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Humoral immune deficiency


Humoral immune deficiencies are conditions which cause impairment of humoral immunity, which can lead to immunodeficiency It can be mediated by insufficient number or function of B cells, the plasma cells they differentiate into, or the antibody secreted by the plasma cells The most common such immunodeficiency is inherited selective IgA deficiency, occurring between 1 in 100 and 1 in 1000 persons, depending on populationThey are associated with increased vulnerability to infection, but can be difficult to detect or asymptomatic in the absence of infectionThey can be associated with increased risk of gastric cancer

Contents

  • 1 Signs and symptoms
  • 2 Causes
  • 3 Diagnosis
  • 4 Treatment
  • 5 See also
  • 6 References
  • 7 Further reading
  • 8 External links

Signs and symptoms

Signs/symptoms of humoral immune deficiency depend on the cause, but generally include signs of infection such as:

  • Sinusitis
  • Sepsis
  • Skin infection
  • Pneumonia

Causes

Cause of this deficiency is divided into primary and secondary:

  • Primary the International Union of Immunological Societies classifies primary immune deficiencies of the humoral system as follows:
Hyper-IgM syndromesimmunoglobulin M
  • Absent B cells with a resultant severe reduction of all types of antibody: X-linked agammaglobulinemia btk deficiency, or Bruton's agammaglobulinemia, μ-Heavy chain deficiency, l 5 deficiency, Igα deficiency, BLNK deficiency, thymoma with immunodeficiency
  • B cells low but present, but with reduction in 2 or more isotypes usually IgG & IgA, sometimes IgM: common variable immunodeficiency CVID, ICOS deficiency, CD19 deficiency, TACI TNFRSF13B deficiency, BAFF receptor deficiency
  • Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgM syndromes
  • Normal numbers of B cells with isotype or light chain deficiencies: heavy chain deletions, kappa chain deficiency, isolated IgG subclass deficiency, IgA with IgG subsclass deficiency, selective immunoglobulin A deficiency
  • Transient hypogammaglobulinemia of infancy THI
  • Secondary secondary or acquired forms of humoral immune deficiency are mainly due to hematopoietic malignancies and infections that disrupt the immune system:
  • Multiple myeloma
  • Chronic lymphoid leukemia
  • AIDS

Diagnosis

Human B cell

In terms of diagnosis of humoral immune deficiency depends upon the following:

  • Measure serum immunoglobulin levels
  • B cell count
  • Family medical history

Treatment

Further information: Immunoglobulin therapy

Treatment for B cell deficiencyhumoral immune deficiency depends on the cause, however generally the following applies:

  • Treatment of infectionantibiotics
  • Surveillance for malignancies
  • Immunoglobulin replacement therapy

See also

  • Immunodeficiency
  • T cell deficiency

References

  1. ^ a b "Pure B-Cell Disorders: Background, Pathophysiology, Epidemiology" 2017-01-06 
  2. ^ a b Notarangelo L, Casanova JL, Conley ME, et al 2006 "Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005" J Allergy Clin Immunol 117 4: 883–96 doi:101016/jjaci2005121347 PMID 16680902 
  3. ^ a b Page 432, Chapter 22, Table 221 in: Jones, Jane; Bannister, Barbara A; Gillespie, Stephen H 2006 Infection: Microbiology and Management Wiley-Blackwell ISBN 1-4051-2665-5 
  4. ^ a b c d Fried, Ari J; Bonilla, Francisco A 2009-07-01 "Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections" Clinical Microbiology Reviews 22 3: 396–414 doi:101128/CMR00001-09 ISSN 0893-8512 PMC 2708392  PMID 19597006 
  5. ^ a b Cecil, Russell La Fayette; Goldman, Lee; Schafer, Andrew I 2012-01-01 Goldman's Cecil Medicine, Expert Consult Premium Edition -- Enhanced Online Features and Print, Single Volume,24: Goldman's Cecil Medicine Elsevier Health Sciences p 1618 ISBN 1437716040 
  6. ^ Pieper, Kathrin; Grimbacher, Bodo; Eibel, Hermann 2013-04-01 "B-cell biology and development" Journal of Allergy and Clinical Immunology 131 4: 959–971 doi:101016/jjaci201301046 ISSN 0091-6749 PMID 23465663 
  7. ^ N Franklin Adkinson Jr; Bochner, Bruce S; Burks, Wesley; Busse, William W; Holgate, Stephen T 2013-11-01 Middleton's Allergy: Principles and Practice Elsevier Health Sciences p 1134 ISBN 9780323085939 

Further reading

  • Ahn, Sam; Cunningham-Rundles, Charlotte 2017-05-11 "Role of B cells in common variable immune deficiency" Expert review of clinical immunology 5 5: 557–564 doi:101586/eci0943 ISSN 1744-666X PMC 2922984  PMID 20477641 
  • Honjo, Tasuku; Reth, Michael; Radbruch, Andreas; Alt, Frederick 2014-10-09 Molecular Biology of B Cells Elsevier ISBN 9780123984906 

External links

Classification V · T · D
  • ICD-10: D80
  • ICD-9-CM: 2790


  • PubMed


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