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==Overview==


In [[medicine]], '''immunodeficiency''' (or '''immune deficiency''') is a state in which the [[immune system]]'s ability to fight [[infectious disease]] is compromised or entirely absent. Most cases of immunodeficiency are acquired ("secondary") but some people are born with defects in the immune system, or [[primary immunodeficiency]]. A person who has an immunodeficiency of any kind is said to be '''immunocompromised'''. An immunocompromised person may be particularly vulnerable to [[opportunistic infection]]s, in addition to normal infections that could affect everyone.


==Primary immunodeficiency==
{{SK}} Immune deficiency; immunity suppression; immunological deficiency; immunosuppression
{{main|Primary immunodeficiency}}
==Overview==
 
Immunodeficiency can be caused by a variety of conditions including autoimmunity, infections, congenital defects, complement defects, or auto-inflammatory states.
A number of [[rare disease]]s feature a heightened susceptibility to [[infection]]s from childhood onward. Many of these disorders are [[genetic disorder|hereditary]] and are [[autosomal recessive]] or [[Sex linkage|X-linked]]. There are over 80 recognised primary immunodeficiency syndromes; they are generally grouped by the part of the immune system that is malfunctioning, such as [[lymphocyte]]s or [[granulocyte]]s.<ref name="pmid7616993">{{cite journal |author=Rosen FS, Cooper MD, Wedgwood RJ |title=The primary immunodeficiencies |journal=[[The New England Journal of Medicine]] |volume=333 |issue=7 |pages=431–40 |year=1995 |month=August |pmid=7616993 |doi=10.1056/NEJM199508173330707 |url=http://www.nejm.org/doi/abs/10.1056/NEJM199508173330707?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed |accessdate=2012-03-08}}</ref>
 
The treatment of primary immunodeficiencies depends on the nature of the defect, and may involve antibody infusions, long-term antibiotics and (in certain cases) [[Hematopoietic stem cell transplantation|stem cell transplantation]].
 
==Acquired immunodeficiency==<!-- This section is linked from [[Immune system]] -->
Immune deficiency may also be the result of particular external processes or diseases; the resultant state is called "secondary" or "acquired" immunodeficiency. Common causes for secondary immunodeficiency are [[malnutrition]], [[aging]] and particular medications (e.g. [[chemotherapy]], [[disease-modifying antirheumatic drug]]s, [[immunosuppressive drug]]s after [[organ transplant]]s, [[glucocorticoid]]s).
 
Many specific diseases directly or indirectly impair the immune system. This include 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 [[AIDS|acquired immunodeficiency syndrome]] (AIDS), caused by the [[HIV|human immunodeficiency virus]] (HIV).  HIV directly attacks the immune system.


==Classification==
<div style="width: 80%; font-size: 85%;">
<small>
{{Family tree/start}}
{{Family tree| | | | | | | | | | | | | | | | | A01 | | | | | | | | |A01=Immunodeficiency}}
{{Family tree| |,|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|+|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|.| }}
{{Family tree| B01 | | B02 | | B03 | | B04 | | B05 | | B06 | | B07 | | B08 | | B09 | |B01=[[Immunodeficiency affecting cellular and humoral Immunity]]|B02=[[Combined immunodeficiency]]|B03=[[Predominantly antibody deficiency]]|B04=[[Diseases of immune dysregulation]]|B05=[[Congenital defects of phagocytes]]|B06=[[Defects in intrinsic and innate immunity]]|B07=[[Auto-inflammatory disorders]]|B08=[[Complement deficiencies]]|B09=[[Phenocopies of primary immunodeficiency]] (PID)}}
{{Family tree|!| | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | | | | | | | |}}
{{Family tree|)| C01 |)| D01 |)| E01 |)| F01 |)| G01 |)| H01 | | | | | | | | | | | | | |C01=Severe combined<br>immunodeficiency (SCID)|D01=Combined immunodeficiency<br>with associated features|E01=Hypogammaglobulinemia|F01=Hemophagocytic lymphohistiocytosis (HLH)<br>& EBV susceptibility|G01=Congenital defects of<br>phagocyte number|H01=Bacterial and<br>parasitic infections}}
{{Family tree|!| | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | | | | | | | |}}
{{Family tree|`| C02 |`| D02 |`| E02 |`| F02 |`| G02 |`| H02 | | | | | | | | | | | | | |C02=Combined immunodeficiencies<br>generally less pronounced than<br>severe combined immunodeficiency|D02=Combined immunodeficiency<br>with syndromic features|E02=Other antibody<br>deficiencies|F02= Syndromes with<br>autoimmunity and others|G02=Congenital defects of<br>phagocyte function|H02=Mendelian susceptibility to<br>mycobacterial disease (MSMD)<br>& viral infections}}
{{Family tree/end}}
</div>
</small>
==References==
==References==
{{reflist|2}}
{{Reflist|2}}


{{Immune_system}}
{{Immune disorders}}
[[Category:Immune system disorders]]
[[Category:Immunodeficiency| ]]
[[Category:Hematology]]
[[Category:Hematology]]
 
[[Category:Medicine]]
[[ca:Immunodeficiència]]
[[Category:Immunology]]
[[de:Immundefekt]]
[[Category:Up-To-Date]]
[[el:Ανοσολογική ανεπάρκεια]]
[[es:Inmunodeficiencia]]
[[fr:Immunodépression]]
[[he:כשל חיסוני]]
[[pt:Imunodeficiência]]
 
{{WH}}
{{WS}}
 
[[Category:Disease]]
[[Category:Infectious disease]]

Latest revision as of 22:21, 29 July 2020

Immunodeficiency
ICD-10 D84.9
ICD-9 279.3
DiseasesDB 21506
MeSH D007153

Immunodeficiency Main Page

Home

Overview

Classification

Immunodeficiency Affecting Cellular and Humoral Immunity

Combined Immunodeficiency

Predominantly Antibody Deficiency

Diseases of Immune Dysregulation

Congenital Defects of Phagocytes

Defects in Intrinsic and Innate Immunity

Auto-inflammatory Disorders

Complement Deficiencies

Phenocopies of Primary Immunodeficiency

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]; Associate Editor(s)-in-Chief: Anmol Pitliya, M.B.B.S. M.D.[3], Ali Akram, M.B.B.S.[4], Zahir Ali Shaikh, MD[5] , Syed Musadiq Ali M.B.B.S.[6], Sabawoon Mirwais, M.B.B.S, M.D.[7]


Synonyms and keywords: Immune deficiency; immunity suppression; immunological deficiency; immunosuppression

Overview

Immunodeficiency can be caused by a variety of conditions including autoimmunity, infections, congenital defects, complement defects, or auto-inflammatory states.

Classification

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Immunodeficiency
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Immunodeficiency affecting cellular and humoral Immunity
 
Combined immunodeficiency
 
Predominantly antibody deficiency
 
Diseases of immune dysregulation
 
Congenital defects of phagocytes
 
Defects in intrinsic and innate immunity
 
Auto-inflammatory disorders
 
Complement deficiencies
 
Phenocopies of primary immunodeficiency (PID)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Severe combined
immunodeficiency (SCID)
 
 
Combined immunodeficiency
with associated features
 
 
Hypogammaglobulinemia
 
 
Hemophagocytic lymphohistiocytosis (HLH)
& EBV susceptibility
 
 
Congenital defects of
phagocyte number
 
 
Bacterial and
parasitic infections
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Combined immunodeficiencies
generally less pronounced than
severe combined immunodeficiency
 
 
Combined immunodeficiency
with syndromic features
 
 
Other antibody
deficiencies
 
 
Syndromes with
autoimmunity and others
 
 
Congenital defects of
phagocyte function
 
 
Mendelian susceptibility to
mycobacterial disease (MSMD)
& viral infections
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

References