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Acquired protein C deficiency

MedGen UID:
388867
•Concept ID:
C2674322
•
Disease or Syndrome
Synonym: Protein C Deficiency, Acquired
SNOMED CT: Acquired protein C deficiency (1217410004)
 
OMIM®: 176860

Professional guidelines

PubMed

Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Heparin-induced thrombocytopenia. Pathogenesis, frequency, avoidance and management.
Warkentin TE
Drug Saf 1997 Nov;17(5):325-41. doi: 10.2165/00002018-199717050-00005. PMID: 9391776

Recent clinical studies

Etiology

Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Laboratory tests for protein C deficiency.
Khor B, Van Cott EM
Am J Hematol 2010 Jun;85(6):440-2. doi: 10.1002/ajh.21679. PMID: 20309856
Human protein C concentrates for replacement therapy in congenital and acquired protein C deficiency.
Knoebl PN
Drugs Today (Barc) 2008 Jun;44(6):429-41. doi: 10.1358/dot.2008.44.6.1217993. PMID: 18596997
Impaired fibrinolysis in the pathogenesis of dengue hemorrhagic fever.
Van Gorp EC, Setiati TE, Mairuhu AT, Suharti C, Cate Ht Ht, Dolmans WM, Van Der Meer JW, Hack CE, Brandjes DP
J Med Virol 2002 Aug;67(4):549-54. doi: 10.1002/jmv.10137. PMID: 12116003
Heparin-induced thrombocytopenia. Pathogenesis, frequency, avoidance and management.
Warkentin TE
Drug Saf 1997 Nov;17(5):325-41. doi: 10.2165/00002018-199717050-00005. PMID: 9391776

Diagnosis

Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Acquired protein C deficiency in a child with acute myelogenous leukemia, splenic, renal, and intestinal infarction.
Farah RA, Jalkh KS, Farhat HZ, Sayad PE, Kadri AM
Blood Coagul Fibrinolysis 2011 Mar;22(2):140-3. doi: 10.1097/MBC.0b013e32834248e6. PMID: 21178585
Laboratory tests for protein C deficiency.
Khor B, Van Cott EM
Am J Hematol 2010 Jun;85(6):440-2. doi: 10.1002/ajh.21679. PMID: 20309856
Heparin-induced thrombocytopenia: a ten-year retrospective.
Warkentin TE
Annu Rev Med 1999;50:129-47. doi: 10.1146/annurev.med.50.1.129. PMID: 10073268
Acquired protein C deficiency in patients with breast cancer receiving cyclophosphamide, methotrexate, and 5-fluorouracil.
Feffer SE, Carmosino LS, Fox RL
Cancer 1989 Apr 1;63(7):1303-7. doi: 10.1002/1097-0142(19890401)63:7<1303::aid-cncr2820630713>3.0.co;2-f. PMID: 2920359

Therapy

Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Use of protein C concentrate in neonatal period.
De Carolis MP
Minerva Pediatr 2010 Jun;62(3 Suppl 1):29-30. PMID: 21089715
Human protein C concentrates for replacement therapy in congenital and acquired protein C deficiency.
Knoebl PN
Drugs Today (Barc) 2008 Jun;44(6):429-41. doi: 10.1358/dot.2008.44.6.1217993. PMID: 18596997
Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans.
Smith OP, White B, Vaughan D, Rafferty M, Claffey L, Lyons B, Casey W
Lancet 1997 Nov 29;350(9091):1590-3. doi: 10.1016/s0140-6736(97)06356-3. PMID: 9393338
Immunopathological events of adverse cutaneous reactions to coumarin and heparin.
Hermes B, Haas N, Henz BM
Acta Derm Venereol 1997 Jan;77(1):35-8. doi: 10.2340/0001555577035038. PMID: 9059674

Prognosis

Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Impaired fibrinolysis in the pathogenesis of dengue hemorrhagic fever.
Van Gorp EC, Setiati TE, Mairuhu AT, Suharti C, Cate Ht Ht, Dolmans WM, Van Der Meer JW, Hack CE, Brandjes DP
J Med Virol 2002 Aug;67(4):549-54. doi: 10.1002/jmv.10137. PMID: 12116003
Low levels of protein C are associated with poor outcome in severe sepsis.
Yan SB, Helterbrand JD, Hartman DL, Wright TJ, Bernard GR
Chest 2001 Sep;120(3):915-22. doi: 10.1378/chest.120.3.915. PMID: 11555529
Protein C levels as a prognostic indicator of outcome in sepsis and related diseases.
Fisher CJ Jr, Yan SB
Crit Care Med 2000 Sep;28(9 Suppl):S49-56. doi: 10.1097/00003246-200009001-00011. PMID: 11007198
Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans.
Smith OP, White B, Vaughan D, Rafferty M, Claffey L, Lyons B, Casey W
Lancet 1997 Nov 29;350(9091):1590-3. doi: 10.1016/s0140-6736(97)06356-3. PMID: 9393338

Clinical prediction guides

Plasma hypercoagulability in the presence of thrombomodulin but not of activated protein C in patients with cirrhosis.
Lebreton A, Sinegre T, Pereira B, Lamblin G, Duron C, Abergel A
J Gastroenterol Hepatol 2017 Apr;32(4):916-924. doi: 10.1111/jgh.13493. PMID: 27421039
Human protein C concentrates for replacement therapy in congenital and acquired protein C deficiency.
Knoebl PN
Drugs Today (Barc) 2008 Jun;44(6):429-41. doi: 10.1358/dot.2008.44.6.1217993. PMID: 18596997
Low levels of protein C are associated with poor outcome in severe sepsis.
Yan SB, Helterbrand JD, Hartman DL, Wright TJ, Bernard GR
Chest 2001 Sep;120(3):915-22. doi: 10.1378/chest.120.3.915. PMID: 11555529
Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans.
Smith OP, White B, Vaughan D, Rafferty M, Claffey L, Lyons B, Casey W
Lancet 1997 Nov 29;350(9091):1590-3. doi: 10.1016/s0140-6736(97)06356-3. PMID: 9393338
Coagulation activation and tissue necrosis in meningococcal septic shock: severely reduced protein C levels predict a high mortality.
Fijnvandraat K, Derkx B, Peters M, Bijlmer R, Sturk A, Prins MH, van Deventer SJ, ten Cate JW
Thromb Haemost 1995 Jan;73(1):15-20. PMID: 7740486

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