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Increased total hemolytic complement activity

MedGen UID:
1637521
•Concept ID:
C4703607
•
Pathologic Function
HPO: HP:0031905

Definition

An abnormally elevated total hemolytic complement activity in the circulation. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIncreased total hemolytic complement activity

Professional guidelines

PubMed

COVID-19 vaccination prevents a more severe course and treatment with complement inhibitors reduce worsening hemolysis during the Omicron pandemic in patients with PNH: a single-center study.
Wang L, Hu Q, Yang Y, Chen M, Yang C, Han B
Ann Med 2023;55(2):2274510. Epub 2024 Jan 1 doi: 10.1080/07853890.2023.2274510. PMID: 38163328Free PMC Article
Recombinant human complement component C2 produced in a human cell line restores the classical complement pathway activity in-vitro: an alternative treatment for C2 deficiency diseases.
Martini PG, Cook LC, Alderucci S, Norton AW, Lundberg DM, Fish SM, Langsetmo K, Jönsson G, Lood C, Gullstrand B, Zaleski KJ, Savioli N, Lottherand J, Bedard C, Gill J, Concino MF, Heartlein MW, Truedsson L, Powell JL, Tzianabos AO
BMC Immunol 2010 Aug 20;11:43. doi: 10.1186/1471-2172-11-43. PMID: 20727163Free PMC Article
Influence of surgical treatment of periapical lesions on serum and blood levels of inflammatory mediators.
Marton IJ, Kiss C
Int Endod J 1992 Sep;25(5):229-33. doi: 10.1111/j.1365-2591.1992.tb01154.x. PMID: 1291518

Recent clinical studies

Etiology

Sutimlimab in Cold Agglutinin Disease.
Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S
N Engl J Med 2021 Apr 8;384(14):1323-1334. doi: 10.1056/NEJMoa2027760. PMID: 33826820
Plasmapheresis in HLA-immunosensitized patients prior to kidney transplantation.
Alarabi A, Backman U, Wikström B, Sjöberg O, Tufveson G
Int J Artif Organs 1997 Jan;20(1):51-6. PMID: 9062832
Increased soluble IL-2 receptor in serum of patients with systemic lupus erythematosus.
Sawada S, Hashimoto H, Iijma S, Tokano Y, Matsukawa Y, Takei M, Ishikawa H, Kang H, Tomura K, Mitamura K
Clin Rheumatol 1993 Jun;12(2):204-9. doi: 10.1007/BF02231527. PMID: 8358979
Determination of C3 nephritic factor activity by a microassay based on the peroxidase-like activity of the heme group.
Marín MA, Domingo A, Fontán G, López-Trascasa M
Clin Biochem 1990 Dec;23(6):497-9. doi: 10.1016/0009-9120(90)80038-k. PMID: 1963123
Immune complexes in multiple sclerosis: relation to clinical pattern.
Patzold U, Haller P, Baruth B, Liman W, Deicher H
J Neurol 1980;222(4):249-60. doi: 10.1007/BF00313154. PMID: 6154784

Diagnosis

Immunodeficiency: Complement disorders.
McMurray JC, Schornack BJ, Weskamp AL, Park KJ, Pollock JD, Day WG, Brockshus AT, Beakes DE, Schwartz DJ, Mikita CP, Pittman LM
Allergy Asthma Proc 2024 Sep 1;45(5):305-309. doi: 10.2500/aap.2024.45.240050. PMID: 39294906Free PMC Article
Diagnostic Utility of Complement Serology for Atypical Hemolytic Uremic Syndrome.
Sridharan M, Go RS, Abraham RS, Fervenza FC, Sethi S, Bryant SC, Spears GM, Murray DL, Willrich MAV
Mayo Clin Proc 2018 Oct;93(10):1351-1362. doi: 10.1016/j.mayocp.2018.07.008. PMID: 30286829
Systemic complement activation and complement gene analysis in enterohaemorrhagic Escherichia coli-associated paediatric haemolytic uraemic syndrome.
Ahlenstiel-Grunow T, Hachmeister S, Bange FC, Wehling C, Kirschfink M, Bergmann C, Pape L
Nephrol Dial Transplant 2016 Jul;31(7):1114-21. Epub 2016 May 4 doi: 10.1093/ndt/gfw078. PMID: 27190382
Expression and localization of serum resistance associated protein in Trypanosoma brucei rhodesiense.
Milner JD, Hajduk SL
Mol Biochem Parasitol 1999 Nov 30;104(2):271-83. doi: 10.1016/s0166-6851(99)00151-6. PMID: 10593181
Determination of C3 nephritic factor activity by a microassay based on the peroxidase-like activity of the heme group.
Marín MA, Domingo A, Fontán G, López-Trascasa M
Clin Biochem 1990 Dec;23(6):497-9. doi: 10.1016/0009-9120(90)80038-k. PMID: 1963123

Therapy

Sutimlimab in Cold Agglutinin Disease.
Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S
N Engl J Med 2021 Apr 8;384(14):1323-1334. doi: 10.1056/NEJMoa2027760. PMID: 33826820
Repeated injection of high doses of hemoglobin-encapsulated liposomes (hemoglobin vesicles) induces accelerated blood clearance in a hemorrhagic shock rat model.
Taguchi K, Iwao Y, Watanabe H, Kadowaki D, Sakai H, Kobayashi K, Horinouchi H, Maruyama T, Otagiri M
Drug Metab Dispos 2011 Mar;39(3):484-9. Epub 2010 Dec 1 doi: 10.1124/dmd.110.036913. PMID: 21123166
Hepatitis C virus-related liver damage is related to cold activation of complement.
Nomura H, Ogo T, Rikimaru N, Ueno S, Kakuno T, Okamoto O, Shiraishi G, Kashiwagi S
J Clin Gastroenterol 1997 Oct;25(3):529-34. doi: 10.1097/00004836-199710000-00010. PMID: 9412971
Plasmapheresis in HLA-immunosensitized patients prior to kidney transplantation.
Alarabi A, Backman U, Wikström B, Sjöberg O, Tufveson G
Int J Artif Organs 1997 Jan;20(1):51-6. PMID: 9062832
Effects of a streptococcal lipoteichoic acid on complement activation in vitro.
Monefeldt K, Tollefsen T
J Clin Periodontol 1993 Mar;20(3):186-92. doi: 10.1111/j.1600-051x.1993.tb00342.x. PMID: 8450083

Prognosis

Complement Components sC5b-9 and CH50 Predict Prognosis in Heart Failure Patients Combined With Hypertension.
Wang W, Cai D
Am J Hypertens 2020 Jan 1;33(1):53-60. doi: 10.1093/ajh/hpz140. PMID: 31429866
Expression and localization of serum resistance associated protein in Trypanosoma brucei rhodesiense.
Milner JD, Hajduk SL
Mol Biochem Parasitol 1999 Nov 30;104(2):271-83. doi: 10.1016/s0166-6851(99)00151-6. PMID: 10593181
Plasmapheresis in HLA-immunosensitized patients prior to kidney transplantation.
Alarabi A, Backman U, Wikström B, Sjöberg O, Tufveson G
Int J Artif Organs 1997 Jan;20(1):51-6. PMID: 9062832
Urinary neopterin in patients with systemic lupus erythematosus.
Leohirun L, Thuvasethakul P, Sumethkul V, Pholcharoen T, Boonpucknavig V
Clin Chem 1991 Jan;37(1):47-50. PMID: 1988207
Immune complexes in multiple sclerosis: relation to clinical pattern.
Patzold U, Haller P, Baruth B, Liman W, Deicher H
J Neurol 1980;222(4):249-60. doi: 10.1007/BF00313154. PMID: 6154784

Clinical prediction guides

DAMPS and complement activation in platelet concentrates that induce adverse reactions in patients.
de Wit YES, Hamzeh-Cognasse H, Cognasse F, Ten Brinke A, Zeerleder SS
Transfusion 2022 Sep;62(9):1721-1726. Epub 2022 Aug 11 doi: 10.1111/trf.17061. PMID: 35950480
Sutimlimab in Cold Agglutinin Disease.
Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S
N Engl J Med 2021 Apr 8;384(14):1323-1334. doi: 10.1056/NEJMoa2027760. PMID: 33826820
Expression and localization of serum resistance associated protein in Trypanosoma brucei rhodesiense.
Milner JD, Hajduk SL
Mol Biochem Parasitol 1999 Nov 30;104(2):271-83. doi: 10.1016/s0166-6851(99)00151-6. PMID: 10593181
Plasmapheresis in HLA-immunosensitized patients prior to kidney transplantation.
Alarabi A, Backman U, Wikström B, Sjöberg O, Tufveson G
Int J Artif Organs 1997 Jan;20(1):51-6. PMID: 9062832
Receptor (CD46) modulation and complement-mediated lysis of uninfected cells after contact with measles virus-infected cells.
Schneider-Schaulies J, Schnorr JJ, Schlender J, Dunster LM, Schneider-Schaulies S, ter Meulen V
J Virol 1996 Jan;70(1):255-63. doi: 10.1128/JVI.70.1.255-263.1996. PMID: 8523534Free PMC Article

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