Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.
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PMID:Myeloproliferative disorders. 105 48

It has been suggested that the ideal definitive treatment of hemorrhage from esophageal varices due to extrahepatic portal hypertension should be a selective shunt, avoiding splenectomy, when possible, for its unfavorable effects. We present a case with extrahepatic portal hypertension and polycythemia vera who complained acute recurrent variceal bleeding in spite of multiple sclerotherapy sessions, with a massive thrombosis of the portal bed and radicles without any possibility to perform a shunt. The combined treatment was emergency splenectomy followed by a closed program of sclerotherapy. The result was good at two years of follow-up. The optimal treatment of patients with episodes of bleeding varices for extrahepatic portal hypertension and patency of splenic vein should be a selective shunt; poor long-term results have been reported in patients without patency of the major tributaries of the portal system and treated with other operations than shunts. Splenectomy could gave a role in producing these unhappy results. After splenectomy it has been observed an increased thrombocytosis, venous infarction of the bowel, increased susceptibility to infection and also a paradoxical higher risk of bleeding by removing low risk collaterals. From this anecdotal report it can be argued that splenectomy is not necessarily followed by bad results and can be the first therapeutic option in emergency and in some selected cases with huge, high risk varices, difficult to treat by sclerosis only, and without suitable vessel for a shunt.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Splenectomy for hemorrhage from esophageal varices with massive thrombosis of the portal bed in the absence of cirrhosis: an intervention to be reevaluated? Reflections on a clinical case]. 175 98

The pathogenesis of portal hypertension arising in patients with myeloproliferative disorders has been difficult to understand because liver biopsy findings often show minimal changes. It has been suggested that increased splenic blood flow, hepatic infiltration with hematopoietic cells or sinusoidal fibrosis may be important. We have reviewed the autopsy findings and clinical histories of 97 patients with polycythemia vera and 48 patients with agnogenic myeloid metaplasia collected from three institutions and from the Polycythemia Vera Study Group. Cirrhosis was present in seven patients, one of whom had bleeding varices. Esophageal varices were present clinically in 10 patients without cirrhosis (seven polycythemia and three agnogenic myeloid metaplasia). All of these patients had lesions in small or medium-sized portal veins and four had stenosis of the extrahepatic portal vein with histology compatible with organized thrombi. Nodular regenerative hyperplasia occurred in 14.6% and correlated closely with the presence of portal vein lesions. Thirty patients had greater than 500 ml of ascites, seven of these patients also had varices and six of them had hepatic vein thrombosis. Ascites also correlated with hepatic vein disease confined to small intrahepatic branches. No correlation was seen between hepatic hematopoietic infiltration and signs of portal hypertension. We conclude that esophageal varices are common and are almost always associated with portal vein lesions visible by light microscopy. These portal vein lesions, and the secondary effects of nodular regenerative hyperplasia and portal hypertension, are most likely a result of portal vein thrombosis in patients with myeloproliferative disorders.
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PMID:Hepatic vascular disease and portal hypertension in polycythemia vera and agnogenic myeloid metaplasia: a clinicopathological study of 145 patients examined at autopsy. 222 15

A patient known to have polycythemia vera developed recurrent melena and was found to have bleeding gastroesophageal varices. Selective superior mesenteric angiography suggested splenic vein thrombosis. Splenectomy led to the disappearance of the varices with no subsequent recurrence of bleeding. We believe this to be the first fully described case of polycythemia vera associated with segmental portal hypertension, and propose that polycythemia vera patients may develop "silent" segmental portal hypertension and that this should be taken into consideration when treating them.
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PMID:Segmental portal hypertension and polycythemia vera. 270 27

The case of a 56-yr-old patient with polycythemia vera who was initially seen with jaundice is reported. Compression of the common bile duct by choledochal varices was found to be the cause of the obstructive jaundice. This compression was successfully treated with placement of a plastic endoprosthesis across the stenosis during endoscopic retrograde cholangiopancreatography. The significance of choledochal varices in the evaluation of obstructive jaundice is emphasized.
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PMID:Compression of the common bile duct due to portal-vein thrombosis in polycythemia vera. 847 63

We report three male patients, with duplex confirmed chronic venous disease, who were on treatment with hydroxyurea for chronic myelogenous leukaemia (CML) and polycythemia vera (PV), referred to us for the management of non-healing perimalleolar ulcers of varying durations. Treatment of the ulcers, based on standard venous ulcer treatment protocols, proved futile, and one patient actually underwent surgery for varicose veins. The ulcers healed only after the discontinuation of hydroxyurea. In another patient, the ulcer continued to progress along with emergence of ankle oedema, venous eczema and lipodermatosclerosis, even after discontinuation of hydroxyurea. The short duration of treatment with the drug before the onset of ulcer and characteristic skin changes of chronic venous disease (CVD), in this patient, made us reconsider our diagnosis and this patient ultimately benefited from varicose veins surgery. Based on our experience with these cases, we have made an attempt to differentiate between the chronic venous ulcer and the drug-induced ulcer on clinical grounds.
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PMID:Perimalleolar ulcers in hydroxyurea treated patients with concomitant chronic venous disease: diagnostic pitfalls. 1981 Mar 13

A 56-year-old female nonsmoker presented with episodic hemoptysis, without any other associated respiratory symptoms. Her medical history was notable for polycythemia rubra vera with portal vein thrombosis, which was treated with warfarin, but was complicated by portal hypertension. Esophageal varices were controlled by endoscopic band ligation. Chest radiograph and 64-slice computed tomography scanning failed to identify a culprit lesion. Bronchoscopy identified a vascular structure in the proximal trachea. A contrast-enhanced 320-multidetector row computed tomography scan of the neck showed a tortuous vascular channel in the trachea, which changed in appearance over time, consistent with a tracheal varix. The patient was changed to aspirin therapy and was evaluated by a cardiothoracic surgeon. A conservative approach was adopted, and the patient has had no recurrence of symptoms.
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PMID:Tracheal varix in portal hypertension. 2135 42

A 75-year old man had been diagnosed at 42 years of age as having polycythemia vera and had been monitored at another hospital. Progression of anemia had been recognized at about age 70, and the patient was thus referred to our center in 2008 where secondary myelofibrosis was diagnosed based on bone marrow biopsy findings. Hematemesis due to rupture of esophageal varices occurred in January and February of 2011. The bleeding was stopped by endoscopic variceal ligation. Furthermore, in March of the same year, hematemesis recurred and the patient was transported to our center. He was in irreversible hemorrhagic shock and died. The autopsy showed severe bone marrow fibrosis with mainly argyrophilic fibers, an observation consistent with myelofibrosis. The liver weighed 1856 g the spleen 1572 g, indicating marked hepatosplenomegaly. The liver and spleen both showed extramedullary hemopoiesis. Myelofibrosis is often complicated by portal hypertension and is occasionally associated with gastrointestinal hemorrhage due to esophageal varices. A patient diagnosed as having myelofibrosis needs to be screened for esophageal/gastric varices. Myelofibrosis has a poor prognosis. Therefore, it is necessary to carefully decide the therapeutic strategy in consideration of the patient's concomitant conditions, treatment invasiveness and quality of life.
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PMID:Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension. 2285 73

Endoscopic variceal sclerotherapy and ligation are standard treatment modalities used for the management of esophageal varices. Reportedly, sclerotherapy and ligation are associated with complications such as hematuria, pulmonary thrombus formation, pleural effusion, renal dysfunction, and esophageal stenosis. However, hemothorax following sclerotherapy and ligation has not yet been reported. We treated a patient who presented with liver cirrhosis and polycythemia vera and later developed hemothorax following the above-mentioned procedures. An 86-year-old man diagnosed with liver cirrhosis due to chronic hepatitis type B and alcohol abuse underwent variceal sclerotherapy using ethanolamine oleate to treat his esophageal varices. Oozing from the esophageal varices continued even after the sclerotherapy procedure; therefore, we performed endoscopic variceal ligation. The patient developed left-sided hemothorax within 24 h after treatment of his varices, and an emergency thoracotomy was performed. A pulmonary ligament of the left lung was bulging and ripping because of mediastinal hematoma, and oozing was noted. Cessation of bleeding was noted after the laceration of the left pulmonary ligament had been sutured. Ours is the first case of hemothorax reported in a patient following an uncomplicated procedure of sclerotherapy and ligation.
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PMID:Hemothorax following Uncomplicated Endoscopic Variceal Sclerotherapy and Ligation for Esophageal Varices. 2903 74

Polycythemia vera (PV) is classified as a myeloproliferative disorder (MPD). Such patients are prone to both thrombotic and hemorrhagic events. Although gastrointestinal (GI) bleeding is not a prominent manifestation of PV, it would be life threatening and necessitating hospital admission and blood transfusion if it occurs. GI hemorrhage in these patients may be due to Aspirin usage, peptic ulcer disease (PUD), acquired Von Willbrand disease, Dieulafoy lesion (DL), Mallory Weiss tear, and esophageal and gastric varices. DL is an enlarged, thick-walled artery in the muscularis mucosa with a small submucosal defect. In this case report, we describe a 65-year-old man with history of PV presented with a massive upper GI bleeding. After a therapeutic endoscopic hemostasis failure and reoccurrence of bleeding during hospital admission, an abdominal computed tomography (CT) was ordered, which revealed an aberrant artery originated from aorta directly into the stomach. An angiographic embolization was considered for the patient, which was successfully performed. Our patient was complicated by splenic infarction due to splenic collateral arteries embolization and the overwhelming thrombotic tendency of the patient himself due to the history of PV. Fortunately, our patient's signs and symptoms responded to supportive therapies and eventually he discharged well.
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PMID:A Rare Cause of Gastrointestinal Bleeding in a 65-Year-Old Man with History of Polycythemia Vera. 3182 26


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