Humoral immune deficiency explained

Humoral immune deficiency
Symptoms:	Sinusitis
Causes:	Absent B cells(primary), Multiple myeloma(secondary)
Diagnosis:	B cell count, Family medical history
Treatment:	Immunoglobulin replacement therapy

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.^[1] The most common such immunodeficiency is inherited selective IgA deficiency, occurring between 1 in 100 and 1 in 1000 persons, depending on population. They are associated with increased vulnerability to infection, but can be difficult to detect (or asymptomatic) in the absence of infection.

Signs and symptoms

Signs/symptoms of humoral immune deficiency depend on the cause, but generally include signs of infection such as:^[2]

• 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:^{[3] [4]}

• 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:^[5]

• Multiple myeloma

• Chronic lymphoid leukemia

• AIDS

Diagnosis

In terms of diagnosis of humoral immune deficiency depends upon the following:^{[6] [7]}

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

Treatment

Treatment for B cell deficiency (humoral immune deficiency) depends on the cause, however generally the following applies:^[6]

• Treatment of infection (antibiotics)
• Surveillance for malignancies
• Immunoglobulin replacement therapy

See also

• Immunodeficiency
• T cell deficiency

Further reading

• Ahn. Sam. Cunningham-Rundles. Charlotte. Charlotte Cunningham-Rundles . 2017-05-11. Role of B cells in common variable immune deficiency. Expert Review of Clinical Immunology. 5. 5. 557–564. 10.1586/eci.09.43. 1744-666X. 2922984. 20477641.
• Book: Molecular Biology of B Cells. Honjo. Tasuku. Reth. Michael. Radbruch. Andreas. Alt. Frederick. 2014-10-09. Elsevier. 9780123984906. en.

External links

• PubMed

Notes and References

1. Pieper. Kathrin. Grimbacher. Bodo. Eibel. Hermann. 2013-04-01. B-cell biology and development. Journal of Allergy and Clinical Immunology. en. 131. 4. 959–971. 10.1016/j.jaci.2013.01.046. 0091-6749. 23465663. free.
2. Book: Middleton's Allergy: Principles and Practice. N. Franklin Adkinson Jr.. Bochner. Bruce S.. Burks. Wesley. Busse. William W.. Holgate. Stephen T.. 2013-11-01. Elsevier Health Sciences. 1134. 9780323085939. en.
3. Notarangelo L, Casanova JL, Conley ME, etal . 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 . 2006 . 16680902 . 10.1016/j.jaci.2005.12.1347. free .
4. 2017-01-06. Pure B-Cell Disorders: Background, Pathophysiology, Epidemiology.
5. https://books.google.com/books?id=iPuvQDcqW88C&pg=PA432 Page 432
6. Fried. Ari J.. Bonilla. Francisco A.. 2009-07-01. Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections. Clinical Microbiology Reviews. en. 22. 3. 396–414. 10.1128/CMR.00001-09. 0893-8512. 2708392. 19597006.
7. Book: Goldman's Cecil Medicine, Expert Consult Premium Edition -- Enhanced Online Features and Print, Single Volume,24: Goldman's Cecil Medicine. Cecil. Russell La Fayette. Goldman. Lee. Schafer. Andrew I.. 2012-01-01. Elsevier Health Sciences. 1618. 978-1437716047. en.