1/7. Unusual retinal vasculitis in a patient with protein s deficiency and systemic toxoplasmosis: a case report.A 28-year old female patient presented with unilateral dropped visual acuity and a central scotoma. Fundus examination and a fluorescein angiogram were suggestive for central retinal vein prethrombosis. Further tests revealed an isolated protein s deficiency for which oral anticoagulation therapy was initiated. Six months later she presented with new symptoms of a right retrobulbar pain on ocular movements. The retinal vessels had normalized with improved visual acuity. Re-evaluation of the fluorescein angiogram taken six months earlier showed an image compatible with unilateral vasculitis. During this six month period persisting high levels of IgG and IgM antibodies against toxoplasma were observed while no signs of other active infection could be found. This report illustrates that in cases of prethrombosis with vasculitis in a young patient, a full medical examination and a thorough investigation of infectious diseases are warranted.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/7. heparin-induced skin necrosis in a patient with end-stage renal failure and functional protein s deficiency.skin ulceration is a well-characterized thrombotic complication of the heparin-induced thrombocytopenia (HIT) syndrome. We present the case of a 73-year-old diabetic woman nearing end-stage renal failure who developed extensive upper thigh, abdominal and buttock ulceration following initiation of subcutaneous heparin for prophylaxis against deep vein thrombosis. A preliminary diagnosis of calciphylaxis was made based on the classical distribution and macroscopic appearance of the ulceration in a patient with end-stage renal failure and secondary hyperparathyroidism. However skin biopsy showed complete absence of calcium deposits in the dermal microvasculature. The presence of extensive microthrombi within dermal vessels prompted serologic testing to detect a prothrombotic state. We identified the combined presence of heparin-dependent platelet activating (HIT) antibodies and functional protein s deficiency. To our knowledge this is the first reported case of a dialysis patient presenting with skin ulceration induced by heparin and protein s deficiency. This case highlights the importance of a skin biopsy and testing for a hypercoaguable state in patients with end-stage renal disease and skin ulceration. We suggest that HIT antibodies should be requested in all dialysis patients presenting with skin ulceration.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/7. calciphylaxis in a patient with rheumatoid arthritis without renal failure and hyperparathyroidism: the possible role of long-term steroid use and protein s deficiency.calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification that is seen almost exclusively in patients with end-stage renal disease and hyperparathyroidism. We report a case of calciphylaxis that may very well be due to a long-term steroid use in a rheumatoid arthritis patient who had neither end-stage renal disease nor hyperparathyroidism. We also discuss the possible role of protein s deficiency as a contributing factor.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/7. Cutaneous necrosis revealing the coexistence of an antiphospholipid syndrome with acquired protein s deficiency, factor v Leiden and hyperhomocysteinemia.We report an exceptional case of cutaneous necrosis due to the coexistence of 4 thrombophilic factors, inherited and acquired. We would like to draw attention to these unrecognized associations. CASE REPORT: A 72-year-old woman was admitted with a 5 month history of necrotic nonhealing, painful ulcer of both legs and recently a purple toe. She had a history of 3 deep venous thromboses of the leg complicated by pulmonary embolism. A skin biopsy of the ulcer and purple toe showed only thrombosis in the dermal vessel. Laboratory findings showed a circulating lupus anticoagulant, positive anticardiolipin antibodies, antinuclear antibodies (1/320 dilution) and an anti Sm. Moreover, activated protein c resistance associated with factor v Leiden mutation and hyperhomocysteinemia was found; protein S was transiently low. With iloprost, oral anticoagulant, vitamin B12 and folic acid, the evolution was good, with healing of ulcer. COMMENTS: cutaneous necrosis can reveal hypercoagulable states, sometimes complex. We find 4 thrombophilic factors in our case, i.e. antiphospholipid antibodies, factor v Leiden, protein s deficiency and hyperhomocysteinemia. This is exceptional but highlights the role of several constitutional and acquired thrombophilic factors in the genesis of thrombosis. Extended protein C pathway disturbances could explain the mechanism that leads to cutaneous necrosis, in this patient, with an antiphospholipid syndrome. This case shows that it is necessary in some circumstances to make a complete hemostatic laboratory search to detect several thrombophilic factors. If they are present they can justify an oral anticoagulant treatment and a familial screening.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/7. Serendipitous diagnosis of protein s deficiency.A 46-year-old male sought periodontal care for a swelling on his right mandibular gingiva. An excisional biopsy revealed a well-differentiated squamous cell carcinoma. Surgical treatment consisted of a right segmental mandibulectomy with ipsilateral right neck dissection and fibular free flap reconstruction. Two days after the surgical procedure, a weakened Doppler signal suggested vascular compromise of the graft. The patient was returned to the operating room where complete thrombosis of the internal jugular vein (recipient vessel) was observed. This event prompted a complete hematological evaluation that disclosed low serum levels of protein S. The patient was started on systemic heparin and local medicinal leeches. A week later, systemic warfarin sodium was added and successfully resolved the vascular compromise of the graft. Two years later, the patient is active and lives a full life with occasional adjustments of warfarin sodium. This case represents the first report on the treatment of gingival carcinoma that led to the serendipitous discovery of an unrelated and unusual systemic condition, protein s deficiency.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/7. cardiopulmonary bypass for a coronary artery bypass graft patient with heterozygous protein c deficiency and protein s deficiency.cardiopulmonary bypass (CPB) poses great risks for hypercoagulable patients and requires management techniques to ensure an optimal outcome free from thrombotic events. This case report reviews perfusion management techniques that may contribute to a safer CPB experience for a patient deficient in both protein C and protein S. A patient with heterozygous protein c deficiency is at increased risk of thrombosis, especially in the venous circulation. Since it is an essential cofactor for activated protein C, deficiency of free protein S is also linked to a hypercoagulable condition. A 52-year-old male presented to our institution with a past medical history of hypercoagulable state, multiple deep vein thromboses, pulmonary embolisms, and stroke. He was scheduled for two-vessel coronary artery bypass graft surgery to be followed by right carotid endarterectomy (RCEA) before discharge. The anesthesia and perfusion teams worked closely together to ensure that fresh frozen plasma (FFP) was given intraoperatively at appropriate times. heparin dose response and protamine dosage was determined with hemostasis management system (HMS) analysis. The closed CPB circuit and cannulae were Carmeda bonded. Rapid autologous priming, along with the use of a hemoconcentrator, kept the hematocrit above 21 during CPB. Zero-balance ultrafiltration and leukocyte depletion were initiated during rewarming to aid in attenuation of the inflammatory response. To conserve coagulation factors, all pump blood was ultrafiltrated post-CPB and returned to the patient. Laboratory samples drawn on postoperative day (POD) one measured normal protein C activity with subnormal protein S activity. On POD six, the patient underwent RCEA and he was discharged on POD eight without complications.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/7. Postinfectious purpura fulminans caused by an autoantibody directed against protein S.OBJECTIVE: To determine the mechanism responsible for idiopathic purpura fulminans, we investigated the procoagulant and anticoagulant pathways in five consecutive patients, four after varicella, and the fifth after a nonspecific infection. methods: Procoagulant and anticoagulant factors, including protein C, protein S, and antithrombin iii, were measured by quantitative or functional assays. Anti-protein S autoantibodies were identified by dot blotting and Western blotting, and quantified serially by enzyme-linked immunosorbent assay. Clinical and laboratory data were collated retrospectively. RESULTS: In each case the disease began 7 to 10 days after the onset of the precipitating infection, with rapidly progressive purpura leading to extensive areas of skin necrosis. The illness was complicated by impaired perfusion of limbs or digits (two patients), peripheral gangrene resulting in an above-knee amputation (one patient), and major organ dysfunction caused by thromboembolic phenomena involving the lungs (two patients), the heart (one patient), or the kidneys (one patient). Protein S levels were virtually undetectable at the time of admission and failed to respond to infusions of fresh frozen plasma, despite correction of other procoagulant and anticoagulant factors. All five children had anti-protein S IgM and IgG autoantibodies, which persisted for less than 3 months after admission. Decline in the anti-protein S IgG antibody concentration was associated with normalization of the plasma protein S levels. CONCLUSIONS: Autoimmune protein s deficiency may be a common mechanism causing postinfectious idiopathic purpura fulminans. Recognition of the pathophysiologic mechanism may provide a rational basis for treatment. Immediate heparinization, infusions of fresh frozen plasma, and, in cases complicated by major vessel thrombosis, the use of tissue-type plasminogen activator may limit thromboembolic complications.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |