Protein C and/or S deficiency presenting as peripheral arterial insufficiency

Abstract

Although protein C and/or S deficiency has frequently been associated with venous thromboembolic events, instances of arterial thromboses have been reported. However, the exact incidence of protein C and/or S deficiency in patients with peripheral arterial insufficiency has not been established. Furthermore, given the lack of adequate studies to define the natural history and angiographic findings of these patients, the treatment has not been well delineated. Therefore, we conducted a prospective study to investigate the prevalence, characteristic angiographic findings and optimal treatments in patients with peripheral arterial insufficiency associated with protein C and/or S deficiency. Between September 2000 and August 2004, 133 patients who presented with peripheral arterial insufficiency underwent hypercoagulability tests before the initiation of any treatments. Of these, 11 patients (8.3%) with protein C and/or S deficiency were included in this study. There were nine males and two females. The ages ranged from 38 years to 72 years (mean 57 years). All patients showed characteristic angiographic findings: long segment thrombotic occlusion of a main peripheral artery without evidence of atherosclerosis or with mild atherosclerotic changes in the aorta and other major arterial trees. Surgical or endovascular procedures were performed in nine patients: bypass graft in four, thrombectomy in four and catheter-directed thrombolysis in one. Conservative treatment with full anticoagulation was performed in two patients. All patients received pre- and post-operative anticoagulation. Except for one amputated case, clinical and vascular laboratory improvements were achieved in 10 patients. Mean follow-up period was 21 months (range 4-45 months). However, one patient, in whom re-vascularization surgery was performed successfully, discontinued warfarin therapy himself at 10 months after surgery, graft occlusion and limb loss occurred at 30 months after surgery. This initial experience suggests that protein C and/or S deficiency may be an independent risk factor for peripheral arterial insufficiency. Patients who present with peripheral arterial insufficiency and protein C and/or S deficiency demonstrate characteristic angiographic findings. Once the diagnosis of protein C and/or S deficiency is made, patients should be treated with life-long anticoagulation.

Popliteal Artery Occlusion in a Young Baseball Athlete

“…12,14 In a recent study by Cho et al, 15 11 of 133 patients (aged between 38 and 72 years) with peripheral arterial insufficiency were found to have levels of protein C and protein S that were less than 60% of normal. All those patients had occlusions in the lower extremities, and 2 with no history of trauma developed popliteal artery occlusion that was described as having "characteristic angiographic findings" of long segmental thrombotic occlusion without clear evidence of atherosclerosis in the main arterial trees.…”

A Case of a Patient With Protein C Deficiency Presenting With Concurrent Thromboses in the Pulmonary Arteries and Innominate Artery

“…1,2 Various methods to classify protein C deficiency exist. 1,2 Various methods to classify protein C deficiency exist.…”

“…1,2 The incidence of arterial thrombosis in patients with protein C deficiency is relatively low compared with that of venous thrombosis. Activated protein C is a vitamin K-dependent protease that transforms the activated factor V into an inactive one, resulting in an anticoagulant effect.…”

Management of complications in surgery of the colon

“…Occult or known malignancy, obesity, age, infirmity, immobility, pregnancy, hypercoagulative disorders (including inflammatory bowel disease) [13], deficiencies in protein C, protein S and antithrombin III, may all result in hypercoagulability and so predispose to thromboembolic events in patients undergoing major colorectal surgical procedures [14].…”