Cases reported "Thrombosis"

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1/59. Regression of large atrial thrombi and coronary neovascularizations with conventional anticoagulation in mitral stenosis--a case report.

    The authors report a case of angiographically documented multiple coronary neovascularizations originating from the left circumflex artery (LCX) and coursing toward multiple thrombi located in the left atrium in a patient with severe mitral stenosis. The thrombi as well as the neovascularizations underwent near-complete resolution with 4 weeks' anticoagulation therapy with warfarin maintaining an international normalization ratio of 3.5. Percutaneous mitral balloon valvuloplasty was performed successfully without complications.
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2/59. Visual disturbance due to carotid artery thrombosis in a patient with familial hypercholesterolemia; response to surgical thrombotectomy.

    A 48 years-old Japanese man suffered from marked xanthomas on ankles, knees, hand fingers, and foot joints due to insufficient control of serum hypercholesterolemia despite low density lipoprotein (LDL-C) absorptive therapy followed by treatment with potent anti-hypercholesterolemic agents. He had undergone surgical resection of xanthoma on the knee, foot and hand finger joints. Treatment with simvastatin returned the serum total cholesterol levels to nearly normal levels, followed by marked fluctuations. He subsequently experienced transient right-visual disturbance, and roentogenographic examination was performed. The patient was diagnosed as right-common carotid artery thrombosis. After the thrombotectomy of the right-common carotid artery, his visual power was markedly improved.
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3/59. Total necrosis of hepatocellular carcinoma due to spontaneous occlusion of feeding artery.

    Spontaneous total necrosis of hepatocellular carcinoma is extremely rare, with only 15 cases reported to date in the English literature, and the involved mechanism remains unresolved. This paper describes a case of spontaneous necrosis of hepatocellular carcinoma in a 70-year-old man with chronic hepatitis. The patient suffered epigastric pain on admission and computed tomography revealed a 4 cm mass with low density in the left lobe of the liver. Fine needle aspiration biopsy revealed a few scattered, naked and irregular nuclei exhibiting nuclear hyperchromasia in the dirty necrotic background, a finding highly suggestive of malignancy. The lobectomized liver revealed a 3.5 cm, well encapsulated, round, and nearly totally necrotic mass. On microscopic examination, the tumor was found to be composed of thick trabeculae of necrotic tumor cells, supporting the diagnosis of hepatocellular carcinoma. After surgery and throughout 13 months of follow up the patient has recovered well.
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4/59. Isolated superior mesenteric artery thrombosis: a rare cause for recurrent abdominal pain in a child.

    A 4-year-old boy was evaluated for recurrent abdominal pain and failure to thrive over a 1-year period in a pediatric subspecialty clinic. Results of the extensive workup mostly were unremarkable. Eventually, imaging studies of the abdominal aorta revealed an isolated thrombosis of the superior mesenteric artery trunk and compensatory hypertrophy of the inferior mesenteric artery. He had been having abdominal angina symptoms and fear of eating. A detailed family history suggested a possible hypercoagulable state. However, an extensive hematologic evaluation did not reveal a recognizable defect that could produce thrombotic events. He was treated by arterial graft bypass surgery and started on conventional anticoagulants. Several months later, he developed repeat, near-total thrombosis of the graft with recurrence of his symptoms. After balloon dilation of the graft and starting him on appropriate anticoagulant maintenance regimen, he had good symptom relief, and the graft remained patent. This presentation was unusually prolonged for the type of vascular problem identified. The possibility of vascular problems in children, therefore, should be considered. Unidentified cause of hereditary clotting tendency is another challenging aspect of this case.
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5/59. Superior mesenteric vein thrombosis presented transient false positivity for lupus anticoagulant under heparin treatment.

    A 24-year-old Japanese man was admitted because of massive haematemesis and melaena with persistent abdominal pain. Markedly bloody ascites and severely oedematous small intestine were recognized, and angiography then revealed superior mesenteric vein thrombosis. After resection of the necrotic small intestine, continuous intravenous infusion of heparin and urokinase was performed. This patient had no familial or personal history of thrombosis. On the 15th day after operation, an initial search for lupus anticoagulant revealed that the prothrombin time (PT) ratio and dilute activated partial thromboplastin time (aPTT) were positive under heparin treatment, without evidence of rheumatic or connective tissue disease. thrombocytopenia was observed with a nearly normocellular bone marrow. A follow-up examination 1 year later still revealed an increased aPTT. However, all tests for antiphospholipid antibodies had been negative including dilute aPTT for about 2 years since the 15th day after operation. These findings suggest that, in this patient, superior mesenteric vein thrombosis has not been associated with primary antiphospholipid syndrome but is probably idiopathic. Positive tests for lupus anticoagulant in the initial period may be unreliable due to heparin treatment.
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6/59. Antithrombin Phe229Leu: a new homozygous variant leading to spontaneous antithrombin polymerization in vivo associated with severe childhood thrombosis.

    There is increasing evidence that serpin conformational alteration caused by single point mutations can be responsible for protein deficiency associated with human diseases. A typical example is the alpha1-antitrypsin deficiency caused by the Z variant carrying a Glu342Lys substitution. Only a few cases of "conformational disease" involving other serpins have been described so far. We investigated a severe antithrombin deficiency in a 13-month-old child with fever and cerebral venous thrombosis. The infant was found to be homozygous for a new antithrombin gene mutation (7396T>C, predicting a Phe229Leu antithrombin variant), and heterozygous for the factor v Leiden mutation. Mild atypical antithrombin deficiency was found in both parents, who were first cousins, asymptomatic, and heterozygous for the same antithrombin gene mutation. The Phe229Leu variant, which does not readily fit into the current classification of antithrombin deficiency, was shown to be a thermolabile antithrombin that spontaneously polymerized in the proband's circulation. This points to a key role for the conserved Phe at position 229, which is near the reactive site loop in a region critical for serpin function and stability. Molecular modeling suggested how the mutation might destabilize this region of the protein and thereby favor reactive site loop insertion and polymerization. This study provides the first direct evidence of antithrombin polymerization in vivo causing antithrombin deficiency and severe thrombotic disease.
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7/59. Left ventricular apical thrombus formation in a patient with suspected tako-tsubo-like left ventricular dysfunction.

    A 74-year-old woman with hypertension and bronchial asthma had chest discomfort at rest and 4 days later was admitted to her nearby hospital because of the sudden onset of right hemiparesis. The hemiparesis had almost disappeared within 24 h of onset, but because an electrocardiogram showed sinus tachycardia and diffuse symmetrical T-wave inversion, she was referred for cardiac examination. coronary angiography did not reveal any significant coronary artery stenosis, but left ventriculography revealed severe hypokinesis of the left ventricular apical region, which contained a 4 x 4-mm solid thrombus moving freely with a wavy motion. Moreover, the activity of both protein c and protein s had decreased. The thrombus disappeared after 2 weeks of anticoagulant treatment with warfarin. Her clinical course suggested that the transient cerebral ischemic attack was caused by embolism of the left ventricular thrombus associated with 'tako-tsubo-like left ventricular dysfunction'.
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8/59. Large left atrial thrombus formation despite warfarin therapy after device closure of a patent foramen ovale.

    Appropriate anticoagulation after transcatheter device placement is controversial. patients with no history of thromboembolism or neurologic event typically receive antiplatelet therapy for several months while the device endothelializes. For patients with a history of stroke, there are no established guidelines for postdevice anticoagulation. Most patients receive warfarin, antiplatelet therapy, or a combination. Thrombus formation after transcatheter device placement has been reported for most commercially available devices. We describe a patient who developed a left atrial thrombus after closure of a patent foramen ovale with a CardioSEAL device. The patient had a normal hypercoaguable laboratory evaluation prior to device placement. Thrombosis occurred despite warfarin therapy before and after device placement. The patient's international normalized ratio was checked every 2 weeks after device placement and ranged between 2.0 and 2.8. She had no clinical arrhythmia during this time period. The left atrial thrombus was detected on routine follow-up transthoracic echocardiogram performed 6 months after device deployment. A subsequent transesophageal echocardiogram demonstrated no residual shunt, appropriate positioning of the device, flat against the septum, and a 1 x 2 cm thrombus attached to the superior and posterior left atrial arm near the junction with the native septum. A fluoroscopic image demonstrated no arm fractures. The device and thrombus were subsequently removed at surgery without complication. This case is perplexing in that the patient received appropriate anticoagulation had a negative hypercoaguable work-up, no residual shunt, and a well-positioned device.
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9/59. Thrombosis in patients with paroxysmal noctural hemoglobinuria is associated with markedly elevated plasma levels of leukocyte-derived tissue factor.

    thromboembolism is a frequent complication of paroxysmal nocturnal hemoglobinuria (PNH) and contributes significantly to patient morbidity and mortality. A number of mechanisms have been proposed to explain the increased incidence of this complication of PNH. Increased platelet activation with platelet microparticle formation and depression of cell surface-mediated fibrinolysis has been demonstrated in patients with PNH. We have studied two patients with hemolytic PNH who had recurrent and refractory venous thromboembolic events despite therapeutic anticoagulation. plasma samples from both patients demonstrated marked hemostatic activation as determined by elevated plasma thrombin-antithrombin complexes (TAT) and D-dimers. plasma samples from both patients were also shown to contain markedly elevated levels of circulating tissue factor (TF), which was shown to be predominantly derived from monocytes and macrophages. In one patient, a successful allogeneic bone marrow transplant resulted in a reduction in hemostatic activation associated with a marked decrease in circulating tissue factor to near normal levels. We propose that thrombosis in PNH results from increased tissue factor expression by complement injured CD55- and CD59-deficient monocytes and macrophages.
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10/59. Left ventricular free-floating ball thrombus complicating aortic valve stenosis.

    A 67-year-old man was referred for evaluation of near-syncopal attacks and left hemiparesis. echocardiography revealed moderate to severe calcific aortic valve stenosis and a free-floating thrombus. Left ventricular (LV) systolic function was normal. No regional wall motion abnormality was detected in the left ventricle. On serial echocardiography, the thrombus began to fragment. Urgent surgery was commenced, during which the mass was seen to be a free-floating ball thrombus in the LV cavity, in addition to apical fibrin bands mimicking abnormal trabeculation. The thrombus was removed and aortic prosthetic valve replacement performed. No coagulation abnormalities were detected. The patient made a full recovery after surgery. Floating thrombus can embolize at any moment and require emergency treatment, notably because of a high mortality rate of systemic embolic events or acute hemodynamic decompensation caused by LV outflow tract obstruction. To the authors' knowledge, this is the first report of LV free-floating thrombus concomitant with isolated calcific aortic valve stenosis.
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