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Decreased circulating total IgA

MedGen UID:
871140
Concept ID:
C4025611
Finding
Synonyms: Decreased total IgA in blood; Total immunoglobulin A deficiency
 
HPO: HP:0003460

Definition

Undetectable serum immunoglobulin A level at a value < 5 mg/dL (0.05 g/L). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDecreased circulating total IgA

Conditions with this feature

Immunodeficiency 18
MedGen UID:
816457
Concept ID:
C3810127
Disease or Syndrome
Immunodeficiency-18 is an autosomal recessive primary immunodeficiency characterized by onset in infancy or early childhood of recurrent infections. The severity is variable, encompassing both a mild immunodeficiency and severe combined immunodeficiency (SCID), resulting in early death without bone marrow transplantation in some patients. Immunologic work-up of the IMD18 SCID patients shows a T cell-negative, B cell-positive, natural killer (NK) cell-positive phenotype, whereas T-cell development is not impaired in the mild form of IMD18 (summary by de Saint Basile et al., 2004).
Immunodeficiency 47
MedGen UID:
934786
Concept ID:
C4310819
Disease or Syndrome
Immunodeficiency-47 (IMD47) is an X-linked recessive complex syndrome characterized by liver dysfunction, recurrent bacterial infections, hypogammaglobulinemia, and defective glycosylation of serum proteins. Some patients also have neurologic abnormalities (summary by Jansen et al., 2016).
Immunodeficiency 70
MedGen UID:
1740270
Concept ID:
C5436501
Disease or Syndrome
Immunodeficiency-70 (IMD70) is an autosomal dominant immunologic disorder characterized by severe cutaneous warts on the hands, feet, and face, suggesting increased susceptibility to human papillomavirus (HPV) infection. Affected individuals may also have recurrent bacterial infections, such as sinusitis, as well as feature of autoinflammation, such as colitis, celiac disease, and retinal vasculitis. Laboratory studies show decreased CD4+ T cells and decreased CD19+ B cells; hypogammaglobulinemia has also been observed (summary by Thaventhiran et al., 2020).
Immunodeficiency 82 with systemic inflammation
MedGen UID:
1781752
Concept ID:
C5543581
Disease or Syndrome
Immunodeficiency-82 with systemic inflammation (IMD82) is a complex autosomal dominant immunologic disorder characterized by recurrent infections with various organisms, as well as noninfectious inflammation manifest as lymphocytic organ infiltration with gastritis, colitis, and lung, liver, CNS, or skin disease. One of the more common features is inflammation of the stomach and bowel. Most patients develop symptoms in infancy or early childhood; the severity is variable. There may be accompanying fever, elevated white blood cell count, decreased B cells, hypogammaglobulinemia, increased C-reactive protein (CRP; 123260), and a generalized hyperinflammatory state. Immunologic workup shows variable B- and T-cell abnormalities such as skewed subgroups. Patients have a propensity for the development of lymphoma, usually in adulthood. At the molecular level, the disorder results from a gain-of-function mutation that leads to constitutive and enhanced activation of the intracellular inflammatory signaling pathway. Treatment with SYK inhibitors rescued human cell abnormalities and resulted in clinical improvement in mice (Wang et al., 2021).

Professional guidelines

PubMed

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Recent clinical studies

Etiology

Borysenko A, Timokhina T, Kononova O
Georgian Med News 2021 Oct;(319):22-27. PMID: 34749317
Jhee JH, Kang HY, Wu M, Nam BY, Chang TI, Jung SY, Park S, Kim H, Yun HR, Kee YK, Yoon CY, Park JT, Yoo TH, Kang SW, Han SH
Clin Chem Lab Med 2017 Nov 27;56(1):75-85. doi: 10.1515/cclm-2017-0090. PMID: 28672768
Simonovska N, Zafirova-Ivanovska B, Babulovska A, Pereska Z, Jurukov I, Trenchevska-Siljanovska L
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Kim SJ, Koo HM, Lim BJ, Oh HJ, Yoo DE, Shin DH, Lee MJ, Doh FM, Park JT, Yoo TH, Kang SW, Choi KH, Jeong HJ, Han SH
PLoS One 2012;7(7):e40495. Epub 2012 Jul 6 doi: 10.1371/journal.pone.0040495. PMID: 22792353Free PMC Article

Diagnosis

Chen XQ, Tu L, Zou JS, Zhu SQ, Zhao YJ, Qin YH
Front Immunol 2021;12:668974. Epub 2021 Sep 3 doi: 10.3389/fimmu.2021.668974. PMID: 34539623Free PMC Article
Okugawa Y, Toiyama Y, Hur K, Yamamoto A, Yin C, Ide S, Kitajima T, Fujikawa H, Yasuda H, Koike Y, Okita Y, Hiro J, Yoshiyama S, Araki T, Miki C, McMillan DC, Goel A, Kusunoki M
J Cachexia Sarcopenia Muscle 2019 Jun;10(3):536-548. Epub 2019 Mar 25 doi: 10.1002/jcsm.12403. PMID: 31091026Free PMC Article
Almeida J, Polvorosa MA, Gonzalez-Quintela A, Madruga I, Marcos M, Pérez-Nieto MA, Hernandez-Cerceño ML, Orfao A, Laso FJ
Alcohol Clin Exp Res 2015 Aug;39(8):1476-84. Epub 2015 Jul 4 doi: 10.1111/acer.12783. PMID: 26146763
Adriaanse MP, Tack GJ, Passos VL, Damoiseaux JG, Schreurs MW, van Wijck K, Riedl RG, Masclee AA, Buurman WA, Mulder CJ, Vreugdenhil AC
Aliment Pharmacol Ther 2013 Feb;37(4):482-90. Epub 2013 Jan 7 doi: 10.1111/apt.12194. PMID: 23289539
Kim SJ, Koo HM, Lim BJ, Oh HJ, Yoo DE, Shin DH, Lee MJ, Doh FM, Park JT, Yoo TH, Kang SW, Choi KH, Jeong HJ, Han SH
PLoS One 2012;7(7):e40495. Epub 2012 Jul 6 doi: 10.1371/journal.pone.0040495. PMID: 22792353Free PMC Article

Therapy

Ferreira Costa AP, Gonçalves AK, Machado PRL, Souza LBFC, Sarmento A, Cobucci RNO, Giraldo PC, Witkin SS
Asian Pac J Cancer Prev 2018 Aug 24;19(8):2313-2317. doi: 10.22034/APJCP.2018.19.8.2313. PMID: 30141308Free PMC Article
Ruiz FS, Andersen ML, Martins RC, Zager A, Lopes JD, Tufik S
Innate Immun 2012 Feb;18(1):44-54. Epub 2010 Nov 18 doi: 10.1177/1753425910385962. PMID: 21088046
Trollmo C, Gudmundsson S, Feltelius N, Rogberg S, Smedegård G, Klareskog L
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Rheumatol Int 1993;13(3):113-6. doi: 10.1007/BF00290298. PMID: 7901887

Prognosis

Borysenko A, Timokhina T, Kononova O
Georgian Med News 2021 Oct;(319):22-27. PMID: 34749317
Ferreira Costa AP, Gonçalves AK, Machado PRL, Souza LBFC, Sarmento A, Cobucci RNO, Giraldo PC, Witkin SS
Asian Pac J Cancer Prev 2018 Aug 24;19(8):2313-2317. doi: 10.22034/APJCP.2018.19.8.2313. PMID: 30141308Free PMC Article
Jhee JH, Kang HY, Wu M, Nam BY, Chang TI, Jung SY, Park S, Kim H, Yun HR, Kee YK, Yoon CY, Park JT, Yoo TH, Kang SW, Han SH
Clin Chem Lab Med 2017 Nov 27;56(1):75-85. doi: 10.1515/cclm-2017-0090. PMID: 28672768
Li XH, Huang XP, Pan L, Wang CY, Qin J, Nong FW, Luo YZ, Wu Y, Huang YM, Peng X, Yang ZH, Liao YH
BMC Nephrol 2016 Nov 2;17(1):164. doi: 10.1186/s12882-016-0378-4. PMID: 27806690Free PMC Article
Kim SJ, Koo HM, Lim BJ, Oh HJ, Yoo DE, Shin DH, Lee MJ, Doh FM, Park JT, Yoo TH, Kang SW, Choi KH, Jeong HJ, Han SH
PLoS One 2012;7(7):e40495. Epub 2012 Jul 6 doi: 10.1371/journal.pone.0040495. PMID: 22792353Free PMC Article

Clinical prediction guides

Goyins KA, Yu JJ, Papp SB, Beddard R, Murthy AK, Chambers JP, Arulanandam BP
Exp Biol Med (Maywood) 2022 Jun;247(12):1055-1060. Epub 2022 Apr 2 doi: 10.1177/15353702221084966. PMID: 35369776Free PMC Article
Borysenko A, Timokhina T, Kononova O
Georgian Med News 2021 Oct;(319):22-27. PMID: 34749317
Jhee JH, Kang HY, Wu M, Nam BY, Chang TI, Jung SY, Park S, Kim H, Yun HR, Kee YK, Yoon CY, Park JT, Yoo TH, Kang SW, Han SH
Clin Chem Lab Med 2017 Nov 27;56(1):75-85. doi: 10.1515/cclm-2017-0090. PMID: 28672768
Kim SJ, Koo HM, Lim BJ, Oh HJ, Yoo DE, Shin DH, Lee MJ, Doh FM, Park JT, Yoo TH, Kang SW, Choi KH, Jeong HJ, Han SH
PLoS One 2012;7(7):e40495. Epub 2012 Jul 6 doi: 10.1371/journal.pone.0040495. PMID: 22792353Free PMC Article
Nieman DC, Nehlsen-Cannarella SL
Sports Med 1991 Mar;11(3):183-201. doi: 10.2165/00007256-199111030-00003. PMID: 2047622

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