Successful therapy with argatroban for superior mesenteric vein thrombosis in a patient with congenital antithrombin deficiency

A 38-year-old woman was admitted with superior mesenteric vein (SMV) thrombosis, which was refractory to anticoagulation therapy. The plasma antithrombin activity was decreased and hardly compensated by concentrated antithrombin preparation due to high consumption rate. However, successful anticoagulation was achieved by administration of direct thrombin inhibitor, argatroban. Family studies of antithrombin activity revealed that she had type I congenital antithrombin deficiency. A novel heterozygous mutation in the gene for antithrombin (single nucleotide T insertion at 7916 and 7917, Glu 272 to stop in exon 4) was identified. Argatroban administration would be effective in the treatment of congenital antithrombin deficiency with SMV thrombosis.     

Unusual presentation of a sarcoid patient: multiple arterial and venous thrombosis with chest lymphadenopathy

We depict a case of a 32 year old Mediterranean man, presenting with pulmonary embolism, and diffuse arterial thrombosis of the lower extremities. CT angiography revealed bilateral pulmonary artery occlusions and a mediastinal lymphadenopathy. Duplex Ultrasound of the lower extremities showed no deep venous thrombosis, but occluded popliteal arteries bilaterally with extension to the right distal superficial femoral artery. Mediastinoscopy with hilar lymph node biopsy showed noncaseating granulomas consistent with sarcoidosis. Thrombophilia profile revealed factor II, MTHFR, and factor XIII gene mutations with markedly elevated homocysteine level of 139 μmol/l. This is an atypical rare case of sarcoidosis presenting with pulmonary embolism and multiple arterial thrombosis. T. J. Rebeiz and R. Mahfouz contributed equally to the work and should both be considered as first authors.     

肠系膜静脉血栓形成和肠系膜静脉硬化症的临床分析

本文报告了肠系膜静脉血栓形成（ＭＶＴ）７例和肠系膜静脉硬化症（ＭＰＳ）２例,就其临床表现,Ｘ线和肉眼病理所见加以叙述。方法回例做肠系膜动脉造影（ＳＭＡ）,５例反复做Ｘ线腹部平片检查,全例最后诊断依剖腹术。结果ＳＭＡ疑ＭＶＴ１例,Ｘ线显示５例肠梗阻,仅２例见扩张的小肠见有肥厚的肠壁襟,外科和病理证实肠梗死是由于血栓形成。来自于肠系膜上静脉和主要分支有７例,而２例是由ＭＰＳ所致,受累的肠管已坏死并肠壁水肿和出血,又肠系膜水肿和增厚,能手术的４例（住院后３６小时内）,３例成活（２例为ＭＰＳ）,从住院到剖腹术延误时间中位数为２．５天,６例死亡（包括１例术后吻合口再栓塞）,死亡率６６．７％。结论ＭＶＴ和ＭＰＳ其临床特征是剧烈腹痛,症状大于体征,早期诊断并对可疑之病人立即剖腹术是最重要的。     

Acute prosthetic valve thrombosis in a patient with neoplastic disease--difficulties with anticoagulation therapy

A case of a 50-year-old woman with prosthetic mitral valve complicated by severe thrombosis as an effect of incorrect anticoagulation treatment during rectal cancer biopsy, is presented. During echocardiographic evaluation a dysfunction prosthetic mitral valve with extremely high maximal mitral gradient (50.2 mmHg) was found. The patient was qualified for surgical intervention and mechanical prosthetic mitral valve was replaced by a biological one. This report underlines the difficulties in the anticoagulation therapy in patients with prosthetic heart valves.     

Recent portal and mesenteric venous thrombosis associated with Fusobacterium bacteremia

SUMMARY: Septic pylephlebitis is usually a complication of intraabdominal infection in the region drained by the portal venous system. We report two cases of portal and mesenteric venous thrombosis associated with Fusobacterium necrophorum bacteremia, which did not show any obvious intra-abdominal source of infection with noninvasive imaging procedures. In one case, early anticoagulation treatment was associated with repermeation of the portal vein and its right branch. As in Bacteroides bacteremia, portal and/or mesenteric venous thrombosis should be searched for in case of Fusobacterium bacteremia of unknown origin. Repermeation of the portal vein and relief of extrahepatic portal hypertension can be achieved in these cases with early anticoagulation.     

Plasma coagulation factor levels in venous thrombosis

High plasma levels of several coagulation factors have been described to be associated with an increased risk of venous thrombosis. However, the mechanisms underlying these associations, as well as those involved in the regulation of plasma levels of coagulation factors, are mostly unknown. Whether these factors should be included in the workup of patients with venous thrombosis remains to be determined. In this review, we discuss the present knowledge on the effects of plasma levels of coagulation factors on the development of venous thrombosis. Furthermore, we review recent findings and ideas on the mechanisms through which elevated plasma coagulation factor levels may influence thrombosis. Finally, we enter into the matter of the possible determinants of elevated plasma levels of coagulation factors.     

Coagulation abnormalities in idiopathic portal venous thrombosis

BACKGROUND: Portal vein thrombosis, usually idiopathic, is the cause of portal hypertension in 46% of Indian patients, who present with a variceal bleed. The presence of lupus anticoagulant (LA) and antithrombin III deficiency have been reported to be associated with an increased tendency to venous thrombosis. METHODS AND RESULTS: We studied 30 patients with portal venous thrombosis diagnosed by ultrasound. Two patients were positive for a lupus anticoagulant and both had very prolonged partial thromboplastin time with kaolin. None of the patients had antithrombin III deficiency.     

急性肠系膜上静脉血栓形成

急性肠系膜上静脉血栓形成 (ＳＭＶ)并不罕见 ,占肠血管病的 5%～ 10 % ,从 1911年至今年已有数百例病例报告 ,其发病率占外科住院病人的 1/ 10 ,0 0 0 ,占急性腹痛剖腹术病人的 1/ 10 0 0 ,平均发病年龄 4 7～ 60岁 ,性别之间无何差别[1] 。病因与发病机理[2～ 3]最常见的病因是高凝固状态 ,包括蛋白Ｃ缺乏、蛋白Ｓ缺乏、Ｖ因子Ｌｅｉｄｅｎ突变、抗凝血酶Ⅲ缺乏和抗心磷脂 (ａｎｔｉｃａｒｄｉｏｌｉｐｉｎ)抗体等 (表 1)。病人常以往有深静脉血栓形成 ,这是导致高凝状态的基础。其次见于高粘度综合征、创伤和感染、少见的原因见于腹内恶…     

Abnormal coagulation and deep venous thrombosis in patients with advanced cancer

Many studies have demonstrated increased coagulation activation in cancer patients and have shown evidence of chronic, low-grade disseminated intravascular coagulation, although most patients remained asymptomatic. In general, patients have not been screened for deep venous thrombosis (DVT). We screened 98 patients with advanced malignancy for DVT using light reflection rheography. Coagulation profiles of DVT and non-DVT groups were studied. We found a high prevalence of DVT (50%) on screening. Overall, the patients had raised levels of fibrinogen (66% patients), factor VIII:C (43%), fragment 1 + 2 (71%) and TAT levels (89%). Patients with DVT had a significantly lower level of fibrinogen than those without (4.0 g/dl, SD 1.4, compared with 4.7 g/dl SD 1.6, P = 0.04). There was no significant difference in other coagulation or liver function tests between the DVT and non-DVT groups. The wide variation of results makes their interpretation difficult and unlikely to be of predictive value in estimating individual thrombotic risk.