A case of Budd-Chiari syndrome secondary to multiple thrombogenic conditions: a case report and review of literature.

This report describes a case of Budd-Chiari syndrome caused by latent polycythemia vera and factor V Leiden mutation. This syndrome usually occurs due to thrombosis of hepatic veins or membranous obstruction of inferior vena cava. The most common reasons for thrombosis are manifest polycythemia vera and the other prothrombotic conditions. Recently, latent polycythemia vera and factor V Leiden mutation have been reported in increasing frequency. In this report, we aimed to emphasize that all prothrombotic conditions must be evaluated while investigating the etiology of Budd-Chiari syndrome, including latent polycythemia vera and factor V Leiden mutation, and appropriate antithrombotic and surgical therapies must be performed without delay.     

Isolated cerebellar infarction as a presenting symptom of polycythemia vera.

Although polycythemia vera is one of the reported causes for cerebral infarction, isolated cerebellar infarction, a rare disorder, was never reported in combination with polycythemia vera. This is a report of a 72-year-old woman in whom isolated cerebellar infarction was the presenting manifestation of polycythemia vera. The patient was treated with recurrent phlebotomies until the hematocrit decreased to < 45%. This treatment was followed by marked neurological improvement. A better awareness of the possibility of cerebellar infarction in polycythemia vera may disclose additional cases.     

Budd-chiari syndrome complicating restorative proctocolectomy for ulcerative colitis

PURPOSE: This is a case of hepatic vein thrombosis presenting in a delayed fashion after proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis. Search for a causative thrombotic condition resulted in the diagnosis of polycythemia vera, a myeloproliferative disorder associated with hypercoagulability. The polycythemia was masked by an iron deficiency associated with the ulcerative colitis. METHODS: The history, physical, diagnostic modalities, and treatment for this patient are described, and the literature of Budd-Chiari syndrome associated with ulcerative colitis is reviewed. RESULTS: Six cases of Budd-Chiari syndrome in the setting of ulcerative colitis are reported in the literature from 1945 to 1997. CONCLUSIONS: Hepatic vein thrombosis is a rare complication of ulcerative colitis. The diagnosis of Budd-Chiari syndrome demands a thorough search for a hematologic condition predisposing to thrombosis. Our patient had a myeloproliferative disorder, polycythemia vera, that is associated with a hypercoagulable state. The disorder was masked by an iron deficiency associated with the ulcerative colitis. Recognition of the entity will permit successful treatment.     

Heparin-associated thrombocytopenia in a patient with polycythemia vera: the importance of a marked drop in platelet count

The present report describes a patient with polycythemia vera who developed a severe arterial and venous thrombosis caused by systemically administered heparin. An immunologic implication has been proposed as pathophysiological mechanism of this heparin-associated thrombocytopenia and thrombosis syndrome. It is suggested, however, that a strong decrease in platelet count as occurred in this patient leads to a higher chance of developing this complication.     

Pulmonary hypertension in polycythemia vera

Thromboembolic events occur in about 27% of the patients with polycythemia vera and account for 31% of the deaths. These include cerebrovascular accidents, myocardial infarction, peripheral vascular occlusions, pulmonary infarctions, and venous thrombosis. We report two cases with polycythemia vera who presented with pulmonary hypertension in the absence of previous thromboembolic complications of any kind. One patient died suddenly, with evidence of extensive bilateral thrombosis of prelobular pulmonary arteries at autopsy. In the second patient, local thrombosis in the pulmonary vasculature or recurrent silent pulmonary emboli appear to be responsible for the development of pulmonary hypertension. After institution of anticoagulant therapy, he is able to maintain his functional status. The purpose of this report is to alert clinicians to the development of this insidious, but potentially fatal complication in patients with polycythemia vera. © 1994 Wiley-Liss, Inc.     

Recurrent spontaneous bacterial peritonitis in a patient with polycythemia vera

Spontaneous bacterial peritonitis (SBP) is an infectious process that usually occurs in patients with cirrhosis. There are few reports of SBP in patients with other pathologies such as nephrotic syndrome, acute and chronic hepatitis, cardiac ascites, and ascites secondary to neoplastic disease. We report a patient with polycythemia vera in whom recurrent episodes of SBP occurred 8 months following a portacaval shunt operation for Budd-Chiari syndrome. Conceivably, the polycythemia vera (PV) complicated by hepatic vein thrombosis and portacaval shunt resulted in significant loss of hepatic reticuloendothelial system function and predisposed the patient to bacterial peritonitis.     

Polycythemia vera--a case report and discussion on pathogenic mechanisms of increased thrombosis

Polycythemia vera is a myeloproliferative disorder characterized by increased red cell mass and frequently complicated by venous and arterial thrombosis. The mechanism underlying the increased incidence of thrombotic events remains illusive. Presented in this report are a case of a 77-year-old man diagnosed with polycythemia vera and a review of the current literature on the mechanisms underlying the increased incidence of thrombotic events in polycythemia vera.     

Polycythemia vera presenting as acute myocardial infarction: An unusual presentation

Acute myocardial infarction (AMI) is usually seen in the setting of atherosclerosis and its associated risk factors. Myocardial infarction in the young poses a particular challenge, as the disease is less likely, due to atherosclerosis. We report the case of a 37-year-old female patient who presented with ST segment elevation anterolateral AMI. The only abnormality on routine blood investigation was raised hemoglobin and hematocrit. After further testing, she was diagnosed according to the World Health Organization (WHO) criteria with polycythemia vera. This case illustrates the importance of recognizing polycythemia vera as an important cause of thrombosis, which can present initially as AMI, and to emphasize the early recognition of the disease in order to initiate appropriate management strategies.     

Localized left atrial administration of tPA for the treatment of mechanical mitral valve thrombosis

Prosthetic valve thrombosis is a dangerous and unfortunately not uncommon medical situation, often seen as a medical emergency. Patients with previously unseen and more confounding medical problems are presenting, making the decision regarding therapeutic options even more complicated. In this case report, we describe a complicated patient with mechanical mitral valve prosthesis placed secondary to rheumatic heart disease, with multiple co‐morbidities including severe left ventricular dysfunction, permanent artrial fibrillation, left femoral deep vein thrombosis, and non‐sustained ventricular tachycardia, who presented with acute valve thrombosis. The patient' history of hemorrhagic stroke and multiple acute ischemic strokes represented an absolute contraindication to more conventional forms of therapy such as intravenous systemic thrombolytics. In the following case report, we present an unprecedented approach to prosthetic valve thrombosis in this seemingly no‐option patient. © 2008 Wiley‐Liss, Inc.     

JAK2 mutation and acute coronary syndrome complicated with stent thrombosis

Acute coronary syndrome (ACS) could be a precious opportunity for patients to reveal concealed diseases other than conventional risk factors for ACS, such as hypertension, dyslipidemia, diabetes mellitus, etc. In the setting of ACS, the intracoronary and systemic prothrombotic environment has led to an increase in the risk of stent thrombosis of which mortality was higher among patients with ACS, especially with the highest mortality in patients with ST elevation myocardial infarction. The some specific conditions which were concealed beyond the cardiovascular pathophysiology except well-known risk factors for ACS and stent thrombosis might involve the onset of ACS. We describe a case of a 64-year-old man who was admitted to intensive care unit for chest pain. This case found the possibility that polycythemia vera with Janus kinase 2 (JAK2) V617F mutation might be a underlying disease of ACS with stent thrombosis, and highlighted the importance of recognizing polycythemia vera with JAK2 V617F mutation as concealed disease for cardiologists. We would like to report and review the relationship between ACS and polycythemia vera with JAK2 V617F mutation.     

Budd-Chiari syndrome with fatal outcome in a patient with polycythemia vera and antiphospholipid antibody syndrome

We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.     

Intraventricular thrombosis in polycythemia vera: A cause of intractable cardiac failure

The occurrence of thrombotic events is central to the course of polycythemia vera.^1–5 Myocardial, cerebral, peripheral, and pulmonary infarctions are frequent and are consequences of thromboses in small and medium caliber arteries. Thrombosis in large caliber arteries is a rare event. Thrombosis within the chambers of the heart has not been hitherto reported.This report documents the occurrence of massive left ventricular thrombosis in a patient with polycythemia vera. The thrombus reduced the left ventricular capacity by about 75% and caused intractable congestive heart failure.     

Erythrocytosis associated with renal artery thrombosis in a patient with polycystic kidney disease on hemodialysis

We report a case of erythrocytosis in a patient with end-stage renal failure on chronic hemodialysis. The patient with polycystic kidney disease had an average Hb level of 10 g/dl while on hemodialysis for 3 years. He developed erythrocytosis (Hb 17.6 g/dl) following a cadaveric renal transplantation. No signs suggesting polycythemia vera were found. Nonrenal causes of secondary erythrocytosis such as anoxia, hemoglobinopathies or tumors were excluded. Angiography showed renal artery occlusion of the native kidney. Serum erythropoietin level was 85 U/l (normal 52 +/- 31 U/l) as measured by 3H-thymidine uptake. It is suggested that ischemia caused by the renal artery thrombosis stimulated the erythropoietin production in the native polycystic kidney.     

Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%, respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.     

Sudden cardiac death caused by native aortic valve thrombosis in an adult patient with a complex congenital cyanotic heart disease

Cyanotic congenital heart disease is associated with an increased risk of thromboembolism. We describe the case of sudden cardiac death caused by native aortic valve thrombosis in adult cyanotic patient. Such embolic complication was not previously described in cyanotic patient. It is also the first report of sudden cardiac death as an initial presentation of the native aortic valve thrombosis. Cyanotic congenital heart disease (CCHD) is associated with an increased risk of thromboembolism due to different haemathologic abnormalities associated with hypoxemia and secondary polycythemia [H. Kajimoto, M. Nakazawa, K. Murasaki, et al. Increased thrombogenesity in patients with cyanotic congenital heart disease. Circ J Jun 2007;71(6):948-953]. Cerebrovascular events, venous and arterial thrombosis have been described in patients with CCHD [N. Ammash, C.A. Warnes. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol Sep 1996;28(3):768-772]. We present a case of the native aortic valve thrombosis in an adult patient with a complex cyanotic congenital heart lesion which led to sudden death.     

Natural history of polycythemia vera and essential thrombocythemia presenting with splanchnic vein thrombosis

Annals of Hematology - Patients with polycythemia vera (PV) or essential thrombocythemia (ET) presenting with splanchnic vein thrombosis (SVT) might have a specific clinico-biological profile. To...     

Occlusion of the superior mesenteric artery in a patient with Polycythemia vera: Resolution with percutaneous transluminal angioplasty

The case is reported of a 46-year-old male patient with polycythemia vera (PV) treated with phlebotomy who developed an occlusive thrombosis of the superior mesenteric artery 2 years after the diagnosis. He was successfully managed with percutaneous transluminal angioplasty. The patient did not develop any other thrombotic phenomena. To our knowledge, there are no previous reports on the use of percutaneous transluminal angioplasty in the management of arterial thrombotic complications in PV patients.     

Splenic rupture complicating periinterventional glycoprotein IIb/IIIa antagonist therapy for myocardial infarction in polycythemia vera

Polycythemia vera is a myeloproliferative disorder predisposing to thromboembolic and bleeding complications. We report the case of a patient with polyglobuly, leukocytosis, and thrombocytosis, who suffered from acute ST-segment elevation myocardial infarction due to thrombotic high-grade pre-stent stenosis two months after percutaneous coronary intervention for complex coronary one vessel disease. Following re-PTCA and stent implantation in conjunction with periinterventional GP IIb/IIIa antagonist treatment, the patient was initially symptom free for about two hours before rapidly developing signs of a hemorrhagic shock. An abdominal CT scan showed splenic rupture with massive intraabdominal hemorrhage as a consequence of secondary bleeding into multiple pre-existing splenic infarctions. The patient's condition stabilized after emergency splenectomy. Subsequent bone marrow biopsy revealed the presence of polycythemia vera. Post-operatively, the patient was treated with the anti-platelet agents aspirin and clopidogrel to prevent subacute stent thrombosis. Additionally, cyto-reductive therapy with hydroxyurea was initiated because of a further increase in the platelet count. In patients with polycythemia vera, the indication for treatment with GP IIb/IIIa antagonists should be carefully weighed against the potentially serious bleeding complications. Should treatment be established, a risk stratification using abdominal sonography and bleeding time testing is recommended, while during treatment red blood count, platelet count, coagulation tests, and hemodynamic parameters should be closely monitored.     

Myocardial infarction and polycythemia vera: how should we treat it?

Little is known about the management and treatment of coronary thrombosis in polycythemia vera (PV) [1]. We describe a case of acute myocardial infarction in a patient with recently diagnosed PV and thrombocytosis. The patient underwent emergency catheterization with intracoronary thrombolysis and angioplasty of the left anterior descending coronary artery. © 1994 Wiley-Liss,Inc..