A Rare Cause of Gastrointestinal Bleeding in a 65-Year-Old Man with History of Polycythemia Vera.
ABSTRACT: 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.
Project description:Objective:To describe the management and prognosis of splenic abscess after splenic arterial embolization in severe acute pancreatitis (SAP) patients. Methods:This is a retrospective observational study. From August 2012 to August 2017, SAP patients with infected pancreatic necrosis (IPN) who underwent splenic arterial embolization after massive hemorrhage of the splenic artery were screened and those who developed splenic abscess were included for analysis. The demographic characteristics, etiology, treatment of splenic abscess, and clinical outcomes of these cases were collected and analyzed. Results:A total of 18 patients with splenic abscess formed after splenic arterial embolization were included for data analysis. The median age of the 18 patients was 46 years. The etiologies included biliary AP, hypertriglyceridemic AP (HTG-AP), and other causes. Ten patients underwent minimally invasive percutaneous drainage only for splenic abscess while the other eight patients received splenectomy. One patient died due to uncontrolled infection and another patient died due to massive bleeding, and the remaining sixteen patients survived. Conclusion:The incidence of splenic abscess was high in patients requiring splenic arterial embolization due to massive bleeding. Our data showed that most splenic abscess could be successfully managed with minimally invasive interventions, and traditional splenectomy should serve as a backup treatment.
Project description:BACKGROUND:Massive gastrointestinal bleeding in children is uncommon. Dieulafoy lesion is an uncommon disease which may lead to massive and repeated upper gastrointestinal hemorrhage. We report two cases of gastric Dieulafoy lesion successfully treated with either band ligation or endoscopic hemoclipping. CASE PRESENTATION:First case report: A previously healthy 18-month-old female infant with E. coli sepsis, pneumonia and respiratory failure with bilateral pneumothorax requiring chest drainage. Over a few days, the patient presented hematemesis and melena with progressively worsening anemia. The esophagogastroduodenoscopy revealed an arterial vessel with eroded apex located between the body and the fundus of the stomach. Two elastic bands were applied which resulted in resolution of hematemesis and melena and improvement of the anemia. Second case report: A 8-year-old male was admitted to our department with sudden massive hematemesis and melena. Clinical examination revealed anemia (hemoglobin, 6.8?g/dl). Esophagogastroduodenoscopy revealed a mucosal erosion with visible vessel located along the small curvature, close to the antrum. Three hemostatic clips were placed on the Dieulafoy lesion and hemostasis was obtained. CONCLUSIONS:we showed that, similar to gastric DL in adult patients,, gastric DL in pediatric patients can be successfully treated with endoscopic therapy, and both hemoclipping and band ligation are suitable techniques.
Project description:Dieulafoy lesions are aberrantly large submucosal arteries most frequently associated with gastrointestinal hemorrhage. They are rarely identified in the bronchial submucosa and can cause massive hemoptysis.We present three episodes of massive hemoptysis in two patients, the first with comorbid Alagille syndrome including multiple cardiac and pulmonary vascular abnormalities and the second with thyroid cancer metastatic to the mediastinum.All episodes were due to Dieulafoy lesions of the bronchus based on bronchoscopic appearance.Bronchoscopic ablation using Nd:YAP laser was attempted both patients.Nd:YAP laser successfully ablated the Dieulafoy lesion in the first case with long-term relief from recurrent hemoptysis. The first episode in the second patient responded to bronchial artery embolization; laser ablation of a different Dieulafoy lesion responsible for the second episode was unsuccessful but additional bronchial artery embolization has provided relief from further episodes.Bronchoscopic ablation of Dieulafoy lesions of the bronchus can provide durable relief from recurrent symptoms. Clinical and anatomical features should be considered carefully before intervention, which should only be attempted by experienced operators with appropriate ancillary support available.
Project description:OBJECTIVES:This study used national audit data to describe current management and outcomes of patients undergoing surgery for complications of peptic ulcer disease (PUD), including perforation and bleeding. It was also planned to explore factors associated with fatal outcome after surgery for perforated ulcers. These analyses were designed to provide a thorough understanding of current practice and identify potentially modifiable factors associated with outcome as targets for future quality improvement. DESIGN:National cohort study using National Emergency Laparotomy Audit (NELA) data. SETTING:English and Welsh hospitals within the National Health Service. PARTICIPANTS:Adult patients admitted as an emergency with perforated or bleeding PUD between December 2013 and November 2015. INTERVENTIONS:Laparotomy for bleeding or perforated peptic ulcer. PRIMARY AND SECONDARY OUTCOME MEASURES:The primary outcome was 60-day in-hospital mortality. Secondary outcomes included length of postoperative stay, readmission and reoperation rate. RESULTS:2444 and 382 procedures were performed for perforated and bleeding ulcers, respectively. In-hospital 60-day mortality rates were 287/2444 (11.7%, 95% CI 10.5% to 13.1%) for perforations, and 68/382 (17.8%, 95% CI 14.1% to 22.0%) for bleeding. Median (IQR) 2-year institutional volume was 12 (7-17) and 2 (1-3) for perforation and bleeding, respectively. In the exploratory analysis, age, American Society of Anesthesiology score and preoperative systolic blood pressure were associated with mortality, with no association with time from admission to operation, surgeon grade or operative approach. CONCLUSIONS:Patients undergoing surgery for complicated PUD face a high 60-day mortality risk. Exploratory analyses suggested fatal outcome was primarily associated with patient rather than provider care factors. Therefore, it may be challenging to reduce mortality rates further. NELA data provide important benchmarking for patient consent and has highlighted low institutional volume and high mortality rates after surgery for bleeding peptic ulcers as a target for future research and improvement.
Project description:A Dieulafoy lesion (DL) of the small bowel can cause severe gastrointestinal bleeding, and presents a difficult clinical setting for endoscopists. Limited data exists on the therapeutic yield of treating DLs of the small bowel using single-balloon enteroscopy (SBE).Data were collected from Tampa General Hospital a 1 018-bed teaching hospital affiliated with University of South Florida in Tampa, Florida. Patients were selected from a database of patients that underwent SBE from January 2010 - August 2013.Eight patients were found to have DL an incidence of 2.6 % of 309 SBE performed for obscure gastrointestinal bleeding. 7/8 were identified in the jejunum, with one found in the duodenum. The mean age of patients with DL was 71.5 years old. 6/8 patients were on some form of anticoagulant/antiplatelet agent. The primary modality of therapy employed was electrocautery, multipolar electrocoagulation in seven patients and APC (argon plasma coagulation) in one patient. In three patients, electrocoagulation was unsuccessful and hemostasis was achieved with clip placement. Three patients required repeat SBE with one found to have rebleeding from a failed clip with hemostasis achieved upon reapplication of one clip.In our United States' experience, SBE offers a reasonable therapeutic approach to treat DL of the small bowel with low rates of rebleeding, no adverse events, and no patient requiring surgery.
Project description:Patients with polycythemia vera (PV), a myeloproliferative neoplasm characterized by an elevated red blood cell mass, are at high risk of vascular and thrombotic complications and have reduced quality of life due to a substantial symptom burden that includes pruritus, fatigue, constitutional symptoms, microvascular disturbances, and bleeding. Conventional therapeutic options aim at reducing vascular and thrombotic risk, with low-dose aspirin and phlebotomy as first-line recommendations for patients at low risk of thrombotic events and cytoreductive therapy (usually hydroxyurea or interferon alpha) recommended for high-risk patients. However, long-term effective and well-tolerated treatments are still lacking. The discovery of mutations in Janus kinase 2 (JAK2) as the underlying molecular basis of PV has led to the development of several targeted therapies, including JAK inhibitors, and results from the first phase 3 clinical trial with a JAK inhibitor in PV are now available. Here, we review the current treatment landscape in PV, as well as therapies currently in development.
Project description:Polycythemia vera (PV) is mainly characterized by elevated blood cell counts, thrombotic as well as hemorrhagic predisposition, a variety of symptoms, and cumulative risks of fibrotic progression and/or leukemic evolution over time. Major changes to its diagnostic criteria were made in the 2016 revision of the World Health Organization (WHO) classification, with both hemoglobin and hematocrit diagnostic thresholds lowered to 16.5 g/dL and 49% for men, and 16 g/dL and 48% for women, respectively. The main reason leading to these changes was represented by the recognition of a new entity, namely the so-called "masked PV", as individuals suffering from this condition have a worse outcome, possibly owing to missed or delayed diagnoses and lower intensity of treatment. Thrombotic risk stratification is of crucial importance to evaluate patients' prognosis at diagnosis. Currently, patients are stratified into a low-risk group, in the case of younger age (<60 years) and no previous thromboses, and a high-risk group, in the case of patients older than 60 years and/or with a previous thrombotic complication. Furthermore, even though they have not yet been formally included in a scoring system, generic cardiovascular risk factors, particularly hypertension, smoking, and leukocytosis, contribute to the thrombotic overall risk. In the absence of agents proven to modify its natural history and prevent progression, PV management has primarily been focused on minimizing the thrombotic risk, representing the main cause of morbidity and mortality. When cytoreduction is necessary, conventional therapies include hydroxyurea as a first-line treatment and ruxolitinib and interferon in resistant/intolerant cases. Each therapy, however, is burdened by specific drawbacks, underlying the need for improved strategies. Currently, the therapeutic landscape for PV is still expanding, and includes several molecules that are under investigation, like long-acting pegylated interferon alpha-2b, histone deacetylase inhibitors, and murine double minute 2 (MDM2) inhibitors.
Project description:Upper gastrointestinal (GI) bleeding is one of the most common, high risk emergency disorders in the western world. Almost nothing has been reported on longer term prognosis following upper GI bleeding. The aim of this study was to establish mortality up to three years following hospital admission with upper GI bleeding and its relationship with aetiology, co-morbidities and socio-demographic factors.Systematic record linkage of hospital inpatient and mortality data for 14 212 people in Wales, UK, hospitalised with upper GI bleeding between 1999 and 2004 with three year follow-up to 2007. The main outcome measures were mortality rates, standardised mortality ratios (SMRs) and relative survival.Mortality at three years was 36.7% overall, based on 5215 fatalities. It was highest for upper GI malignancy (95% died within three years) and varices (52%). Compared with the general population, mortality was increased 27-fold during the first month after admission. It fell to 4.3 by month four, but remained significantly elevated during every month throughout the three years following admission. The most important independent prognostic predictors of mortality at three years were older age (mortality increased 53 fold for people aged 85 years and over compared with those under 40 years); oesophageal and gastric/duodenal malignancy (48 and 32 respectively) and gastric varices aetiologies (2.8) when compared with other bleeds; non-upper GI malignancy, liver disease and renal failure co-morbidities (15, 7.9 and 3.9); social deprivation (29% increase for quintile V vs I); incident bleeds as an inpatient (31% vs admitted with bleeding) and male patients (25% vs female).Our study shows a high late as well as early mortality for upper GI bleeding, with very poor longer term prognosis following bleeding due to malignancies and varices. Aetiologies with the worst prognosis were often associated with high levels of social deprivation.
Project description:<h4>Background</h4>There are limited series concerning Dieulafoy disease of the bronchus. We describe the clinical presentation of a series of 7 patients diagnosed with Dieulafoy disease of the bronchus and provide information about the pathological diagnosis approach.<h4>Patients and methods</h4>A retrospective review of patients who underwent surgery for massive and unexplained recurrent hemoptysis in a referral center during a 11-year period.<h4>Results</h4>Seven heavy smoker (49 pack years) patients (5 males) mean aged 54 years experienced a massive hemoptysis (350-1000 ml) unrelated to a known lung disease and frequently recurrent. Bronchial contrast extravasation was observed in 3 patients, combining both CT scan and bronchial arteriography. Efficacy of bronchial artery embolization was achieved in 40% of cases before surgery. Pathological examination demonstrated a minute defect in 3 cases and a large and dysplasic superficial bronchial artery in the submucosa in all cases.<h4>Conclusion</h4>Dieulafoy disease should be suspected in patients with massive and unexplained episodes of recurrent hemoptysis, in order to avoid hazardous endoscopic biopsies and to alert the pathologist if surgery is performed.
Project description:A 53-year-old male patient with a history of hepatocellular carcinoma developed gastroesophageal varices refractory to endoscopic injection sclerotherapy (EIS). He required EIS six times in 2 years for recurring variceal bleeding. After hepatic resection, he developed massive splenomegaly. Partial splenic embolization (PSE) was performed to reduce the portal pressure. Varices and variceal bleeding were not detected during 13-year follow up, until the patient died of hepatocellular carcinoma. This is a unique case of gastroesophageal varices controlled by PSE and improved portal hypertension.