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Immunodeficiency due to a late component of complement deficiency

MedGen UID:
585067
•Concept ID:
C0398765
•
Disease or Syndrome
Synonyms: deficiency of complement of terminal pathway; immunodeficiency due to a C5 to C9 component complement deficiency; immunodeficiency due to a late component of complement deficiency; Immunodeficiency due to C5 to C9 component complement deficiency; immunodeficiency due to C5 to C9 component complement deficiency; Terminal complement pathway deficiency; terminal complement pathway deficiency; Terminal component deficiency
SNOMED CT: Terminal component deficiency (234608003)
 
Monarch Initiative: MONDO:0015700
Orphanet: ORPHA169150

Definition

Immunodeficiency due to a late component of complement deficiency is a primary immunodeficiency due to an anomaly in either complement components C5, C6, C7, C8 or C9 and is typically characterized by meningitis due to often recurrent meningococcal infections. The prognosis is generally favorable. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Immunodeficiency due to a late component of complement deficiency

Recent clinical studies

Etiology

Multi-component meningococcal serogroup B (MenB)-4C vaccine induces effective opsonophagocytic killing in children with a complement deficiency.
van den Broek B, van Els CACM, Kuipers B, van Aerde K, Henriet SS, de Groot R, de Jonge MI, Langereis JD, van der Flier M
Clin Exp Immunol 2019 Dec;198(3):381-389. Epub 2019 Oct 1 doi: 10.1111/cei.13368. PMID: 31487400Free PMC Article
Deficiencies and excessive human complement system activation in disorders of multifarious etiology.
Tichaczek-Goska D
Adv Clin Exp Med 2012 Jan-Feb;21(1):105-14. PMID: 23214307
Antibody-dependent killing of meningococci by human neutrophils in serum of late complement component-deficient patients.
Platonov AE, Vershinina IV, Käyhty H, Fijen CA, Würzner R, Kuijper EJ
Int Arch Allergy Immunol 2003 Apr;130(4):314-21. doi: 10.1159/000070219. PMID: 12740533
Vaccination and the role of capsular polysaccharide antibody in prevention of recurrent meningococcal disease in late complement component-deficient individuals.
Andreoni J, Käyhty H, Densen P
J Infect Dis 1993 Jul;168(1):227-31. doi: 10.1093/infdis/168.1.227. PMID: 8515116

Diagnosis

Approach to primary immunodeficiency.
Devonshire AL, Makhija M
Allergy Asthma Proc 2019 Nov 1;40(6):465-469. doi: 10.2500/aap.2019.40.4273. PMID: 31690396
Chapter 27: Approach to primary immunodeficiency.
Uzzaman A, Fuleihan RL
Allergy Asthma Proc 2012 May-Jun;33 Suppl 1:91-95. doi: 10.2500/aap.2012.33.3560. PMID: 22794700
Complement C7 deficiency presenting as recurrent aseptic meningitis.
Corvini M, Randolph C, Aronin SI
Ann Allergy Asthma Immunol 2004 Aug;93(2):200-5. doi: 10.1016/S1081-1206(10)61476-7. PMID: 15328683
Recurrent meningococcal infections in a patient with congenital C5 deficiency.
Bols A, Janssens J, Peetermans W, Stevens E, Bobbaers H
Acta Clin Belg 1993;48(1):42-7. doi: 10.1080/17843286.1993.11718283. PMID: 8388602

Therapy

Multi-component meningococcal serogroup B (MenB)-4C vaccine induces effective opsonophagocytic killing in children with a complement deficiency.
van den Broek B, van Els CACM, Kuipers B, van Aerde K, Henriet SS, de Groot R, de Jonge MI, Langereis JD, van der Flier M
Clin Exp Immunol 2019 Dec;198(3):381-389. Epub 2019 Oct 1 doi: 10.1111/cei.13368. PMID: 31487400Free PMC Article
Vaccination and the role of capsular polysaccharide antibody in prevention of recurrent meningococcal disease in late complement component-deficient individuals.
Andreoni J, Käyhty H, Densen P
J Infect Dis 1993 Jul;168(1):227-31. doi: 10.1093/infdis/168.1.227. PMID: 8515116

Prognosis

Recurrent meningococcal infections in a patient with congenital C5 deficiency.
Bols A, Janssens J, Peetermans W, Stevens E, Bobbaers H
Acta Clin Belg 1993;48(1):42-7. doi: 10.1080/17843286.1993.11718283. PMID: 8388602

Clinical prediction guides

Multi-component meningococcal serogroup B (MenB)-4C vaccine induces effective opsonophagocytic killing in children with a complement deficiency.
van den Broek B, van Els CACM, Kuipers B, van Aerde K, Henriet SS, de Groot R, de Jonge MI, Langereis JD, van der Flier M
Clin Exp Immunol 2019 Dec;198(3):381-389. Epub 2019 Oct 1 doi: 10.1111/cei.13368. PMID: 31487400Free PMC Article
Deficiencies and excessive human complement system activation in disorders of multifarious etiology.
Tichaczek-Goska D
Adv Clin Exp Med 2012 Jan-Feb;21(1):105-14. PMID: 23214307
Antibody-dependent killing of meningococci by human neutrophils in serum of late complement component-deficient patients.
Platonov AE, Vershinina IV, Käyhty H, Fijen CA, Würzner R, Kuijper EJ
Int Arch Allergy Immunol 2003 Apr;130(4):314-21. doi: 10.1159/000070219. PMID: 12740533

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