We report a case of a 6-year-old girl with severe protein S deficiency due to a homozygous mutation and recurrent episodes of skin necrosis. She developed purpura fulminans at birth and a catheter-related venous thrombosis complicated by massive pulmonary embolism at the sixth day of life. Long-term oral anticoagulant therapy with a vitamin K-antagonist was started with a therapeutic range of the international normalized ratio of prothrombin time between 2.0 and 3.0. Unfortunately, this common range was not sufficient because recurrent episodes of warfarin-induced skin necrosis developed if the international normalized ratio was <4.0. Vitamin K antagonists decrease plasma level of vitamin K–dependent coagulation proteins, including the natural anticoagulant protein C. In our patient, the hypercoagulable state due to warfarin-induced reduction of protein C, other than severe protein S deficiency, outweighed the anticoagulant efficacy of the inhibition of procoagulant factors II, VII, IX, and X. The switch of anticoagulant therapy from warfarin to rivaroxaban, a direct inhibitor of activated factor X that does not inhibit other vitamin K–dependent proteins, resulted in the disappearance of skin necrosis at 1 year of follow-up. Rivaroxaban may be considered as a valid anticoagulant alternative in patients with severe inherited protein S deficiency and warfarin-induced skin necrosis.
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November 2013
Case Report|
November 01 2013
Anticoagulant Treatment With Rivaroxaban in Severe Protein S Deficiency
Ida Martinelli, MD;
aA. Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, and
Address correspondence to: Ida Martinelli, MD, PhD, Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Via Pace, 9, 20122 Milan, Italy. E-mail: martin@policlinico.mi.it
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Paolo Bucciarelli, MD;
Paolo Bucciarelli, MD
aA. Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, and
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Andrea Artoni, MD;
Andrea Artoni, MD
aA. Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, and
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Emilio F. Fossali, MD;
Emilio F. Fossali, MD
bDepartment of Maternal and Pediatric Sciences, Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico and University of Milan, Milan, Italy
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Serena M. Passamonti, MD;
Serena M. Passamonti, MD
aA. Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, and
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Armando Tripodi, PhD;
Armando Tripodi, PhD
aA. Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, and
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Flora Peyvandi, MD
Flora Peyvandi, MD
aA. Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, and
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Address correspondence to: Ida Martinelli, MD, PhD, Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Via Pace, 9, 20122 Milan, Italy. E-mail: martin@policlinico.mi.it
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
Pediatrics (2013) 132 (5): e1435–e1439.
Article history
Accepted:
June 18 2013
Citation
Ida Martinelli, Paolo Bucciarelli, Andrea Artoni, Emilio F. Fossali, Serena M. Passamonti, Armando Tripodi, Flora Peyvandi; Anticoagulant Treatment With Rivaroxaban in Severe Protein S Deficiency. Pediatrics November 2013; 132 (5): e1435–e1439. 10.1542/peds.2013-1156
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