Immunodeficiency

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Immunodeficiency

Other names Immunocompromisation, immune deficiency

Specialty Immunology

Immunodeficiency, also known as immunocompromisation, is a state in

which the immune system's ability to fight infectious diseases and cancer is
compromised or entirely absent. Most cases are acquired ("secondary") due to
extrinsic factors that affect the patient's immune system. Examples of these
extrinsic factors include HIV infection and environmental factors, such
as nutrition.[1] Immunocompromisation may also be due to genetic diseases/flaws.
An example here is SCID.
In clinical settings, immunosuppression by some drugs, such as steroids, can
either be an adverse effect or the intended purpose of the treatment. Examples
of such use is in organ transplant surgery as an anti-rejection measure and in
patients suffering from an overactive immune system, as in autoimmune
diseases. Some people are born with intrinsic defects in their immune system,
or primary immunodeficiency.[2]
A person who has an immunodeficiency of any kind is said to
be immunocompromised. An immunocompromised individual may particularly
be vulnerable to opportunistic infections, in addition to normal infections that
could affect anyone.[3] It also decreases cancer immunosurveillance, in which the
immune system scans the body's cells and kills neoplastic ones.

Contents

 1Types
o 1.1By affected component
o 1.2Primary or secondary
 1.2.1Primary immunodeficiency
 1.2.2Secondary immunodeficiencies
 2Immunodeficiency and autoimmunity
 3Causes
 4Treatment
 5Prognosis
 6See also
 7References
 8External links

Types[edit]
By affected component[edit]

Humoral immune deficiency (including B cell deficiency or dysfunction),
with signs or symptoms depending on the cause, but generally include signs
of hypogammaglobulinemia (decrease of one or more types of antibodies)
with presentations including repeated mild respiratory infections,
and/or agammaglobulinemia (lack of all or most antibody production) which
results in frequent severe infections and is often fatal. [4]

T cell deficiency, often causes secondary disorders such as acquired
immune deficiency syndrome (AIDS).[5]

Granulocyte deficiency, including decreased numbers
of granulocytes (called as granulocytopenia or, if absent, agranulocytosis)
such as of neutrophil granulocytes (termed neutropenia). Granulocyte
deficiencies also include decreased function of individual granulocytes, such
as in chronic granulomatous disease.

Asplenia, where there is no function of the spleen

Complement deficiency is where the function of the complement system is
deficient

In reality, immunodeficiency often affects multiple components, with notable

examples including severe combined immunodeficiency (which is primary)
and acquired immune deficiency syndrome (which is secondary).

Comparison of immunodeficiencies by affected component

Affected Main pathogens of resultant

Main causes[6]
components infections[6]

Humoral  Primary humoral  Streptococcus pneumoniae

immune  Multiple myeloma  Hemophilus influenzae
deficiency B cells, plasma  Chronic lymphoid  Pneumocystis jirovecii
cells or antibodies leukemia  Giardia intestinalis
B cell
deficiency  AIDS  Cryptosporidium parvum
  Marrow and
other transplantation
 AIDS Intracellular pathogens,
T cell  Cancer chemotherapy including Herpes simplex
T cells
deficiency  Lymphoma virus, Mycobacterium, Listeria,[7] and
intracellular fungal infections.[6]
 Glucocorticoid therapy
 Chemotherapy  Enterobacteriaceae
 Bone marrow  Oral Streptococci
transplantation  Pseudomonas aeruginosa
Neutrophil  Enterococcus species
Neutropenia
granulocytes  Candida species
 Dysfunction, such
as chronic granulomatous
disease  Aspergillus species
 Polysaccharide encapsulated
bacteria,[8] particularly:
o
Streptococcus
pneumoniae[8]
 Splenectomy o
Haemophilus
 Trauma
Asplenia Spleen influenzae[8]
o
Neisseria
 Sickle-cell anemia meningitidis[8]
 Plasmodium species

 Babesia species
 Neisseria species
Complement Complement
 Congenital deficiencies
deficiency system
 Streptococcus pneumoniae

Primary or secondary[edit]
The distinction between primary versus secondary immunodeficiencies is based
on, respectively, whether the cause originates in the immune system itself or is,
in turn, due to insufficiency of a supporting component of it or an external
decreasing factor of it.
Primary immunodeficiency[edit]
Main article: Primary immunodeficiency
A number of rare diseases feature a heightened susceptibility to infections from
childhood onward. Primary Immunodeficiency is also known as congenital
immunodeficiencies.[9] Many of these disorders are hereditary and are autosomal
recessive or X-linked. There are over 95 recognised primary immunodeficiency
syndromes; they are generally grouped by the part of the immune system that is
malfunctioning, such as lymphocytes or granulocytes.[10]
The treatment of primary immunodeficiencies depends on the nature of the
defect, and may involve antibody infusions, long-term antibiotics and (in some
cases) stem cell transplantation. The characteristics of lacking and/or impaired
antibody functions can be related to illnesses such as X-Linked
Agammaglobulinemia and Common Variable Immune Deficiency [11]
Secondary immunodeficiencies[edit]
Further information: Immunosuppression
Secondary immunodeficiencies, also known as acquired immunodeficiencies,
can result from various immunosuppressive agents, for
example, malnutrition, aging, particular medications
(e.g., chemotherapy, disease-modifying antirheumatic
drugs, immunosuppressive drugs after organ transplants, glucocorticoids)
and environmental toxins like mercury and other heavy
metals, pesticides and petrochemicals like styrene, dichlorobenzene, xylene,
and ethylphenol. For medications, the term immunosuppression generally refers
to both beneficial and potential adverse effects of decreasing the function of the
immune system, while the term immunodeficiency generally refers solely to the
adverse effect of increased risk for infection.
Many specific diseases directly or indirectly cause immunosuppression. This
includes many types of cancer, particularly those of the bone marrow and blood
cells (leukemia, lymphoma, multiple myeloma), and certain chronic infections.
Immunodeficiency is also the hallmark of acquired immunodeficiency
syndrome (AIDS),[9] caused by the human immunodeficiency virus (HIV). HIV
directly infects a small number of T helper cells, and also impairs other immune
system responses indirectly.
Various hormonal and metabolic disorders can also result in immune deficiency
including anemia, hypothyroidism and hyperglycemia.
Smoking, alcoholism and drug abuse also depress immune response.

Immunodeficiency and autoimmunity[edit]

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There are a large number of immunodeficiency syndromes that present clinical

and laboratory characteristics of autoimmunity. The decreased ability of the
immune system to clear infections in these patients may be responsible for
causing autoimmunity through perpetual immune system activation. [12]
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One example is common variable immunodeficiency (CVID) where multiple

autoimmune diseases are seen, e.g., inflammatory bowel disease,
autoimmune thrombocytopenia, and autoimmune thyroid disease. Familial
hemophagocytic lymphohistiocytosis, an autosomal recessive primary
immunodeficiency, is another example. Low blood levels of red blood cells, white
blood cells, and platelets, rashes, lymph node enlargement, and enlargement of
the liver and spleen are commonly seen in these patients. Presence of multiple
uncleared viral infections due to lack of perforin are thought to be responsible. In
addition to chronic and/or recurrent infections many autoimmune diseases
including arthritis, autoimmune hemolytic anemia, scleroderma and type 1
diabetes are also seen in X-linked agammaglobulinemia (XLA). Recurrent
bacterial and fungal infections and chronic inflammation of the gut and lungs are
seen in chronic granulomatous disease (CGD) as well. CGD is caused by a
decreased production of [nicotinamide adenine dinucleotide phosphate]]
(NADPH) oxidase by neutrophils. Hypomorphic RAG mutations are seen in
patients with midline granulomatous disease; an autoimmune disorder that is
commonly seen in patients with granulomatosis with polyangiitis and NK/T cell
lymphomas. Wiskott–Aldrich syndrome (WAS) patients also present with
eczema, autoimmune manifestations, recurrent bacterial infections and
lymphoma. In autoimmune polyendocrinopathy-candidiasis-ectodermal
dystrophy (APECED) also autoimmunity and infections coexist: organ-specific
autoimmune manifestations (e.g., hypoparathyroidism and adrenocortical failure)
and chronic mucocutaneous candidiasis. Finally, IgA deficiency is also
sometimes associated with the development of autoimmune and atopic
phenomena. Gene therapy in Immunodeficiency

Causes[edit]
The cause of immunodeficiency varies depending on the nature of the disorder.
The cause can be either genetic or acquired by malnutrition and poor sanitary
conditions.[13][14] Only for some genetic causes, the exact genes are known. [15]

Treatment[edit]
Available treatment falls into two modalities: treating infections and boosting the
immune system.
Prevention of Pneumocystis pneumonia using trimethoprim/sulfamethoxazole is
useful in those who are immunocompromised. [16] In the early 1950s
Immunoglobulin(Ig) was used by doctors to treat patients with primary
immunodeficiency through intramuscular injection. Ig replacement therapy are
infusions that can be either subcutaneous or intravenously administrated,
resulting in higher Ig levels for about three to four weeks, although this varies
with each patient.[11]

Prognosis[edit]
Prognosis depends greatly on the nature and severity of the condition. Some
deficiencies cause early mortality (before age one), others with or even without
treatment are lifelong conditions that cause little mortality or morbidity. Newer
stem cell transplant technologies may lead to gene based treatments of
debilitating and fatal genetic immune deficiencies. Prognosis of acquired immune
deficiencies depends on avoiding or treating the causative agent or condition
(like AIDS).

See also[edit]
 Acquired immune deficiency syndrome (AIDS)
 Immune disorder
o Autoimmune disease, immune response to self-proteins
o Allergy, immune response to harmless non-self proteins
 Histamine
 Immunosenescence, age-associated immune deficiency
 Steroids, commonly administered drugs like prednisone that suppress the
immune system
 Human genetic enhancement
 Immune system
 Immunology

References[edit]
1. ^ Chinen J, Shearer WT (February 2010).  "Secondary immunodeficiencies, including
HIV infection".  The Journal of Allergy and Clinical Immunology. 125 (2 Suppl 2): S195–
203.  doi:10.1016/j.jaci.2009.08.040.  PMC 6151868. PMID 20042227.
2. ^ "Primary immunodeficiency". Mayo Clinic. 30 January 2020. Retrieved 13
May 2020.
3. ^ Meidani, Mohsen; Naeini, Alireza Emami; Rostami, Mojtaba; Sherkat, Roya; Tayeri,
Katayoun (March 2014). "Immunocompromised patients: Review of the most common
infections happened in 446 hospitalized patients".  Journal of Research in Medical
Sciences.  19  (Suppl 1): S71–S73.  ISSN  1735-1995. PMC  4078380.  PMID  25002900.
4. ^ Greenberg S. "Immunodeficiency". University of Toronto. Archived from the
originalon 10 July 2013.
5. ^ Schwartz RA (2019-10-22). Jyonouchi H (ed.). "T-cell Disorders".  Medscape.
6. ^ Jump up to:a b c If not otherwise specified in boxes, then reference for entries
is: Page 432, Chapter 22, Table 22.1 in: Jones J, Bannister BA, Gillespie SH
(2006).  Infection: Microbiology and Management. Wiley-Blackwell. ISBN 978-1-4051-2665-6.
7. ^ Page 435 in: Jones J, Bannister BA, Gillespie SH (2006).  Infection: Microbiology
and Management. Wiley-Blackwell. ISBN 978-1-4051-2665-6.
8. ^ Jump up to:a b c d Brigden ML (February 2001). "Detection, education and
management of the asplenic or hyposplenic patient". American Family Physician. 63 (3):
499–506, 508. PMID 11272299.
9. ^ Jump up to:a b Basic Immunology: Functions and Disorders of the Immune System,
3rd Ed. 2011.
10. ^ Rosen FS, Cooper MD, Wedgwood RJ (August 1995). "The primary
immunodeficiencies". The New England Journal of Medicine.  333  (7): 431–
40.  doi:10.1056/NEJM199508173330707. PMID 7616993.
11. ^ Jump up to:a b "Immune Deficiency Foundation". primaryimmune.org.
Retrieved 2017-04-17.
12. ^ Grammatikos AP, Tsokos GC (February 2012).  "Immunodeficiency and
autoimmunity: lessons from systemic lupus erythematosus". Trends in Molecular
Medicine. 18 (2): 101–
8. doi:10.1016/j.molmed.2011.10.005.  PMC 3278563. PMID 22177735.
13. ^ "Nutrition and Immunity". The Nutrition Source. Harvard T.H. Chan School of Public
Health. Retrieved  8 November  2020.
14. ^ Bourke CD, Berkley JA, Prendergast AJ (2016). "Immune Dysfunction as a Cause
and Consequence of Malnutrition". Trends in Immunology. 37 (6): 386–
398.  doi:10.1016/j.it.2016.04.003.  PMC 4889773. PMID 27237815. Retrieved  10 May2020.
15. ^ Immunobiology: The Immune System in Health and Disease. 5th edition., figure
11.8
16. ^ Stern A, Green H, Paul M, Vidal L, Leibovici L (October 2014).  "Prophylaxis for
Pneumocystis pneumonia (PCP) in non-HIV immunocompromised patients".  The Cochrane
Database of Systematic Reviews.  10  (10):
CD005590. doi:10.1002/14651858.CD005590.pub3.  PMC 6457644. PMID 25269391.