Immunodeficiency: Difference between revisions

Jump to navigation Jump to search
m (Bot: Removing from Primary care)
 
(17 intermediate revisions by 4 users not shown)
Line 12: Line 12:
}}
}}
{{ID}}
{{ID}}
{{CMG}}; {{AE}} {{ZAS}}, {{Akram}}, {{Anmol}}
{{CMG}} {{shyam}}; {{AE}} {{Anmol}}, {{Akram}}, {{ZAS}} , {{Sali}}, {{Sab}}




Line 18: Line 18:
{{SK}} Immune deficiency; immunity suppression; immunological deficiency; immunosuppression
{{SK}} Immune deficiency; immunity suppression; immunological deficiency; immunosuppression
==Overview==
==Overview==
Immunodeficiency can be caused by a variety of conditions including autoimmunity, infections, congenital defects, complement defects, or auto-inflammatory states.


==Classification==
==Classification==
<div style="width: 80%; font-size: 85%;">
<small>
{{Family tree/start}}
{{Family tree/start}}
{{Family tree| | | | | | | | | | | | | | | | | A01 | | | | | | | | |A01=Immunodeficiency}}
{{Family tree| | | | | | | | | | | | | | | | | A01 | | | | | | | | |A01=Immunodeficiency}}
{{Family tree| |,|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|+|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|.| }}
{{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-imflammatory disorders|B08=Complement deficiencies|B09=Phenocopies of primary immunodeficiency (PID)}}
{{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}}
{{Family tree/end}}
 
</div>
===Immunodeficiency Affecting Cellular and Humoral Immunity===
</small>
{{Family tree/start}}
{{Family tree | | | | | | | | | | A01 | | | | | | | | | | | | | | |A01=Immunodeficiency affecting cellular and humoral immunity}}
{{Family tree | | | | | |,|-|-|-|-|^|-|-|-|-|.| | | | | | | | | | |}}
{{Family tree | | | | | B01 | | | | | | | | B02 | | | | | | | | | |B01=CD19 NL: SCID T-ve B+ve|B02=CD19 ↓: SCID T-ve B-ve}}
{{Family tree | | |,|-|-|^|-|-|.| | | |,|-|-|^|-|-|.| | | | | | | |}}
{{Family tree | | C01 | | | | C02 | | C03 | | | | C04 | | | | | | |C01=SCID T-ve B+ve NK-ve|C02=SCID T-ve B+ve NK+ve|C03=SCID T-ve B-ve NK-ve|C04=SCID T-ve B-ve NK+ve}}
{{Family tree | |!| | | | | |!| | | |!| | | | | |,|^|-|-|-|.| | | |}}
{{Family tree | |)| D01 | | |)| E01 |)| F01 | | G01 | | | G02 | | |D01=yc deficiency|E01=IL7Ra .|F01=ADA def|G01=Microcephaly present|G02=Microcephaly absent}}
{{Family tree | |!| | | | | |!| | | |!| | | | |!| | | | |!| | | | |}}
{{Family tree | |`| D02 | | |)| E02 |`| F02 | |)| H01 | |)| H05 | |D02=JAK-3 def|E02=CD3D, CD3E, CD247|F02=Reticular dysgenesis|H01= DNA Ligase IV def|H05=RAG1/2 def}}
{{Family tree | | | | | | | |!| | | | | | | | |!| | | | |!| | | | |}}
{{Family tree | | | | | | | |)| E03 | | | | | |)| H02 | |`| H06 | |E03=CD45 def|H02=XLF def|H06=DCLRE1C def}}
{{Family tree | | | | | | | |!| | | | | | | | |!| | | | | | | | | |}}
{{Family tree | | | | | | | |)| E04 | | | | | |`| H03 | | | | | | |E04=Coronin-1A def|H03=DNA PKcs def}}
{{Family tree | | | | | | | |!| | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | |`| E05 | | | | | | | | | | | | | | | |E05=Winged helix def}}
{{Family tree/end}}
 
===Combined Immunodeficiency===
{{Family tree/start}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | | | | A01 | | | | | | | | | |A01=Combined Immunodeficiency Diseases with associated or syndromic features}}
{{Family tree | | | |,|-|-|-|-|-|-|v|-|-|-|-|-|v|-|-|-|-|-|v|-|-|-|-|+|-|-|-|-|v|-|-|-|-|v|-|-|-|-|v|-|-|-|-|.| |}}
{{Family tree | | | B01 | | | | | B02 | | | | B03 | | | | B04 | | | B05 | | | B06 | | | B07 | | | B08 | | | B09 | | | | | | | | | | | | | |B01=Congenital thromocytopenia|B02=DNA Repair Defects|B03=Immuno-osseous dysplasias|B04=Thymic Defects with additional congenital anomalies|B05=Hyper-IgE syndromes(HIES)|B06=Dyskeratosis congenita (DKC)|B07=Defects of Vitamin B12 and Folate metabolism|B08=Anhidrotic Ectodermodysplasia with ID|B09=Others}}
{{Family tree | | |!| | | | | | |!| | | | | |!| | | | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | |}}
{{Family tree | | |)| C01 | | | |)| D01 | | |)| E01 | | |)| F01 | |)| G01 | |)| H01 | |)| I01 | |)| J01 | |)| K01 | | | | | | |C01=Wiskott Aldrich Syndrome|D01=Ataxia telangiectasia|E01=Cartilage Hair Hypoplasia|F01=DiDeorge Syndrome|G01=Job Syndrome|H01=Dyskeratosis congenita|I01=Transcobalmin 2 deficiency|J01=NEMO deficiency|K01=Purine nucleoside phosphorylase deficiency}}
{{Family tree | | |!| | | | | | |!| | | | | |!| | | | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | }}
{{Family tree | | |)| C02 | | | |)| D02 | | |)| E02 | | |)| F02 | |)| G02 | |)| H02 | |)| I02 | |`| J02 | |)| K02 | | | | | | | |C02=XL thrombocytopenia|D02=Nijmegen breakage Syndrome|E02=Schimke Syndrome|F02=TBX1 deficiency|G02=Comel Netherton Syndrome|H02= COATS plus syndrome|I02=Deficiency causing hereditary folate malabsorption|J02=EDA-ID due to IKBA GOF mutation|K02=ID with multiple intestinal atresias}}
{{Family tree | | |!| | | | | | |!| | | | | |!| | | | | |!| | | | |!| | | | |!| | | | |!| | | | | | | | | |!| | | | | | | | | }}
{{Family tree | | |)| C03 | | | |)| D03 | | |)| E03 | | |)| F03 | |`| G03 | |)| H03 | |`| I03 | | | | | | |)| K03 | | | | | | | | |C03=WIP deficiency|D03=Bloom syndrome|E03=MYSM1 deficiency|F03=Chromosome 10p13-p14 deletion Syndrome|G03=PGM3 deficiency|H03=SAMD9|I03=Methylene-tetrahydrofolate-dehydrogenase 1 deficiency|K03=Hepatic veno-occlusive disease with immunodeficiency}}
{{Family tree | | |!| | | | | | |!| | | | | |!| | | | | |!| | | | | | | | | |!| | | | | | | | | | | | | | |!| | | | | | | | | | | }}
{{Family tree | | |`| C04 | | | |)| D04 | | |)| E04 | | |`| F04 | | | | | | |`| H04 | | | | | | | | | | | |)| K04 | | | | | | | | | |C04=ARPC1B deficiency|D04=PMS2 deficiency|E04=MOPD1 deficiency|H04=SAMD9L|K04=Vici Syndrome}}
{{Family tree | | | | | | | | | |!| | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | |}}
{{Family tree | | | | | | | | | |)| D05 | | |`| E05 | | | | | | | | | | | | | | | | | | | | | | | | | | | |)| K05| | | | | | |D05=Immunodeficiency with centromeric instability and facial anomalies(ICF1, ICF2, ICF3, ICF4)|E05=EXTL3 deficiency|K05=HOIL1 deficiency, HOIP1 deficiency}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |!| | | | |}}
{{Family tree | | | | | | | | | |)| D06 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |)| K06 | | | | |D06=MCM4 deficiency|K06=Calcium Channel Defects(ORAI-1 deficiency, STIM1 deficiency)}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | |}}
{{Family tree | | | | | | | | | |)| D07 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |)| K07 | | | | | | |D07=RNF168 deficiency|K07=Hennekam-lymphangiectasia-lymphedema syndrome}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | |}}
{{Family tree | | | | | | | | | |)| D08 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |)| K08 | | | | | | |D08=POLE1 deficiency|K08=STAT5b deficiency}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | |}}
{{Family tree | | | | | | | | | |)| D09 | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |`| K09 | | | | | | |D09=POLE2 deficiency|K09=Kabuki Syndrome}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | |)| D10 | | | | | | | | | | | | | | | | | | | | | | | |D10=NSMCE3 deficiency}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | |)| D11 | | | | | | | | | | | | | | | | | | | | | | | |D11=ERCC6L2(Hebo deficiency)}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | |)| D12 | | | | | | | | | | | | | | | | | | | | | | | |D12=Ligase 1 deficiency}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | |`| D13 | | | | | | | | | | | | | | | | | | | | | | | |D13=GINS1 deficiency}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |}}
{{Family tree/end}}
 
===Predominantly Antibody Deficiency===
 
===Diseases of Immune Dysregulation===
 
===Congenital Defects of Phagocytes===
 
===Defects in Intrinsic and Innate Immunity===
 
===Auto-imflammatory Disorders===
 
===Complement Deficiencies===
 
===Phenocopies of Primary Immunodeficiency (PID)===
 
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


[[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]]

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