The relative risk of ischemic cerebrovascular accident in patients with Von Willebrand disease.

Cerebrovascular accident (CVA) is one of the leading causes of death in the United States. Von Willebrand factor plays an important role in platelet activation and adhesion. It remains unclear whether Von Willebrand disease (vWD) is associated with a decreased risk of developing CVA. The study aimed to compare the relative risk (RR) of CVA in patients with and without vWD. We queried the National Inpatient Sample from 2009 to 2014 for discharge data and records for vWD and CVA using International Classification of Diseases, Ninth-Revision codes. The unadjusted and adjusted RR of CVA in patients with and without vWD were estimated using log-binomial model. Descriptive measures including means, medians, standard deviations, and range were presented based on normality test of continuous data. The prevalence of CVA was lower in patients with vWD than in those without vWD (1.31% vs 2.04%), with a RR of 0.64 (95% confidence interval (CI): 0.60-0.68). After adjusting for common CVA risk factors, the RR remained lower in vWD patients: 0.81 (95% CI: 0.76-0.86). vWD is associated with a lower RR of developing CVA. This suggests that deficiency of Von Willebrand factor is potentially protective against the development of CVA. To the best of our knowledge, this is the first study in humans to compare the RR of CVA in patients with and without vWD. Future studies are needed to explore causal relationships and therapeutic benefits.

The Cardioprotective Potential of von Willebrand Disease in Ischemic Heart Disease.

von Willebrand factor (vWF) aids coagulation at sites of vessel injury. Elevated vWF levels have been associated with an increased risk of ischemic heart disease (IHD); however, it is unclear whether vWF deficiency, seen in patients with von Willebrand disease (vWD), protects people against IHD. We determined and compared the prevalence and risk of IHD in patients with versus without vWD by using data from the National Inpatient Sample (2009-2014), excluding patients younger than 18 and older than 75 years. The primary outcome was the odds ratio (OR) of IHD in patients with versus without vWD. Secondary outcomes were major medical comorbidities and demographic characteristics in patients with vWD. Of 224,475,443 weighted hospital-discharge samples, we identified 82,809 patients with a vWD diagnosis. The odds of IHD were lower in patients with vWD than in those without (OR=0.54; 95% CI, 0.52-0.56). After multivariable logistic regression analysis and adjustment for age, sex, and typical IHD risk factors (hypertension, smoking, diabetes, hyperlipidemia, chronic kidney disease, obesity, and family history of IHD), the likelihood of IHD remained lower in patients with vWD than in patients without (OR=0.65; 95% CI, 0.63-0.67). Our study shows that vWF deficiency, as seen in patients with vWD, is associated with a decreased prevalence of IHD. Further investigation may confirm these findings.

Constrictive Pericarditis 5 Months after Radiation Therapy in a 62-Year-Old Woman with Esophageal Cancer.

Most pericardial changes appear within a few weeks in patients who have undergone radiation therapy for thoracic neoplasms. Chronic pericardial constriction typically occurs decades later, consequent to fibrosis. Early constrictive pericarditis after chest irradiation is quite rare. We report the case of a 62-year-old woman who underwent radiation therapy for esophageal cancer and presented with constrictive pericarditis 5 months later. We searched the English-language medical literature from January 1986 through December 2015 for reports of early constrictive pericarditis after irradiation for thoracic malignancies. We defined "early" as a diagnosis within one year after radiation therapy. Five cases fit our criteria, and we summarize the findings here. To our knowledge, ours is the first definitive report of a patient with esophageal cancer to present with early radiation-induced constrictive pericarditis. We conclude that constrictive pericarditis can occur early after radiation for thoracic malignancies, albeit rarely. When planning care for cancer patients, awareness of this sequela is helpful.

Extramedullary Cardiac Multiple Myeloma-A Case Report and Contemporary Review of the Literature.

Multiple myeloma (MM) is characterized by a clonal proliferation of plasma cells. Although the bone marrow is the usual site of involvement, extramedullary plasmacytomas (EMPs) also occur, affecting any tissue. Cardiac and pericardial involvement, although described, have been rare occurrences. We present the case of a 61-year-old female patient 47 days after autologous stem cell transplant for MM who developed cardiac tamponade owing to extramedullary recurrence of myeloma, pulmonary embolism, and takotsubo cardiomyopathy. We performed a review of the published studies of all cases of MM presenting at diagnosis or relapse with cardiac or pericardial involvement in the past 25 years. Including our patient, 34 patients with plasmacytoma involving cardiac or pericardial structures were identified from the literature search. Approximately equal numbers of patients were male and female (42% and 57%, respectively). The mean age was 62 years. Primary plasmacytomas accounted for 12% of the cases. A history of MM, EMP, or monoclonal gammopathy of uncertain significance was noted in two thirds of the cases (66.6%). Treatment included chemotherapy and/or high-dose corticosteroids in 81.1% of cases and 27% underwent radiation therapy. The reporting of all cases to date has focused on unusual findings, rather than treatment approaches or new therapeutic strategies that might benefit patients. We suggest the formation of a database of all cases of cardiac and pericardial EMPs, with a focus on predictive disease variables, standardized staging, outcomes, and survival, to ensure that patients are optimally treated in the modern era.