Activated protein C concentrate treatment for skin necrosis under warfarin treatment in severe genetic protein C deficiency combined with prothrombin mutation and factor V Leiden

Naomi Rosenzweig; Tzipora Strauss; Marina Rubinstein; Gideon Paret; Gili Kenet

doi:10.1160/TH08-09-0585

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2009; 101(02): 405-407
DOI: 10.1160/TH08-09-0585

Case Report

Schattauer GmbH

Activated protein C concentrate treatment for skin necrosis under warfarin treatment in severe genetic protein C deficiency combined with prothrombin mutation and factor V Leiden

Authors

Naomi Rosenzweig

¹The Department of Pediatric Critical Care, Safra Children‘s Hospital, Sheba Medical Center, Tel Hashomer, Israel

²Pediatric Coagulation Service, National Hemophillia Center, Sheba Medical Center, Tel Hashomer, Israel
Tzipora Strauss

²Pediatric Coagulation Service, National Hemophillia Center, Sheba Medical Center, Tel Hashomer, Israel
Marina Rubinstein

¹The Department of Pediatric Critical Care, Safra Children‘s Hospital, Sheba Medical Center, Tel Hashomer, Israel
Gideon Paret

¹The Department of Pediatric Critical Care, Safra Children‘s Hospital, Sheba Medical Center, Tel Hashomer, Israel
Gili Kenet

²Pediatric Coagulation Service, National Hemophillia Center, Sheba Medical Center, Tel Hashomer, Israel

Abstract

Full Text

References

References
1 Griffin JH, Evatt B, Zimmerman TS. et al. Deficiency of protein C in congenital thrombotic disease. J Clin Invest 1981; 68: 1370-1373.
2 Hoshi S, Hijikata M, Togashi Y. et al. Protein C deficiency in a family with thromboembolism and identified gene mutations. Intern Med 2007; 46: 997-1003.
3 Marlar RA, Neumann A. Neonatal purpura fulminans due to homozygous protein C or protein S deficiencies. Semin Thromb Hemost 1990; 16: 299-309.
4 Colman RW, Marder VJ, Clowes AW. et al. Hemostasis & Thrombosis. Lippincot-Williams–Wilkins; 5th edition 2005
5 Seligsohn U, Griffin J. Hereditary thrombophilia. Williams Hematology. 7th ed. Mcgraw Hill Medical; NY: 2006
6 Hartman K, Manco-Johnson MJ, Rawlings JS. et al. Homozygous protein C deficiency: early treatment with warfarin. Am J Pediatr Hematol Oncol 1989; 11: 395-401.
7 Monagle P, Andrew M, Halton J. et al. Homozygous protein C deficiency: description of a new mutation and successful treatment with low molecular weight heparin. Thromb Haemost 1998; 79: 756-761.
8 Zimbelman J, Lefkowitz J, Schaeffer C. et al. Unusual complications of warfarin therapy: Skin necrosis and priapism. J Pediatr 2000; 137: 266-268.
9 Fernandez-Burriel M. Severe clinical presentation of protein C deficiency in a type I/II compound heterozygote newborn. Thromb Haemost 2005; 94: 216-218.
10 Dreyfus M, Masterson M, David M. et al. Replacement therapy with a monoclonal antibody purified protein C concentrate in newborns with severe congenital protein C deficiency. Semin Thromb Hemost 1995; 21: 371-381.
11 Bernard GR, Vincent JL, Laterre PF. et al. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Engl J Med 2001; 344: 699-709.
12 Manco-Johnson MJ, Knapp-Clevenger R. Activated protein C concentrate reverses purpura fulminans in severe genetic protein C deficiency. J Pediatr Hematol Oncol 2004; 26: 25-27.
13 Nowak-Gottl U, Bidlingmaier C, Krumpel A. et al. Pharmacokinetids, efficacy, and safety of LMWHs in venous thrombosis and stroker in neonates, infants and children. Br J Pharmacol 2007; 153: 1120-1127.
14 O’brein SH, Lee H, Ritchey AK. Once-daily enoxaparin in pediatric thromboembolism: a dose finding and pharmacodynamics/pharmacokinetics study . J Thromb Haemost 2007; 5: 1985-1987.
15 Meijer P, Haverkate F, Kluft C. Performance goals for the laboratory testing of antithrombin, protein C and protein S. Thromb Haemost 2006; 96: 584-589.
16 Heeb MJ, Espana F, Griffin JH. Inhibition of and complexation of activated protein C by two major inhibitors in plasma. Blood 1989; 73: 446-454.
17 Monagle P, Newall F, Ignjatovic V. et al. Homozygous protein C deficiency: subcutaneous protein C replacement is effective as salvage or maintenance therapy. Blood 2002; 100: 267a.
18 Petäjä J, Fernández JA, Gruber A. et al. Anticoagulant synergism of heparin and activated protein C in vitro. J Clin Invest 1997; 99: 2655-2663.