Sarcoidosis is a granulomatous disease involving multiple organ systems. In its classic form, sarcoidosis is associated with non-caseating granuloma. Several differential diagnostic entities exist ...for sarcoidosis, including tuberculosis (TB), as well as lymphomas. In this report, we present a case of sarcoidosis in a 55-year-old male with diffuse lymphadenopathy and hepatosplenic involvement, highlighting the differential diagnostic point for this disease.
Ampullary carcinoma is an extremely rare type of gastrointestinal cancer that originates at the ampulla of Vater, distal to the junction between the pancreatic duct and the common bile duct (CBD). ...There are three subtypes depending on the histological findings: pancreatobiliary, intestinal, and mixed subtype. Symptoms can mimic other pathologies related to biliary obstruction, such as jaundice, diarrhea, steatorrhea, and weight loss. In this report, we present a case of a 40-year-old male who presented with painless jaundice and dizziness. Magnetic resonance cholangiopancreatography (MRCP) showed choledocholithiasis and CBD dilatation. Endoscopic ultrasound showed a 24 x 14 mm ampulla mass. Subsequently, he underwent the Whipple procedure that revealed an intestinal-type periampullary adenocarcinoma characterized as stage III (T3bN2M0), with lymphovascular and perineural invasion. He was lost to follow-up but was later found to have metastatic pancreatic adenocarcinoma to the lung and liver. In this report, we also discuss the clinical presentation, pathogenesis, and current evidence-based therapeutic options in the management of this tumor, highlighting the importance of treatment choice depending on the tumor type.
The association between malignancies and autoimmunity had been well-established. The proposed pathophysiology and causality can be bidirectional. For example, a paraneoplastic syndrome can be ...triggered by an underlying malignancy or vice versa, where chronic inflammation of organs affected by autoimmunity can induce malignant transformation such as the case with inflammatory bowel disease and colorectal cancer or primary sclerosing cholangitis and hepatobiliary cancer. This report presents a case of autoimmune phenomena, namely, autoimmune hemolytic anemia, pernicious anemia, and Graves disease associated with newly diagnosed breast cancer. We also highlight the postulated pathophysiologic mechanisms in an attempt to answer the question of whether the occurrence of these autoimmune phenomena in our patient is a result of the law of parsimony (Occam's razor), where clinical variables are pathogenically related, or the counterargument, where random events and diseases can take place simultaneously (Hickam's dictum).
Lymphangioleiomyomatosis (LAM) is a rare disorder of abnormal proliferation of smooth muscle-like cells which results in the formation of thin-walled cysts and progressive lung destruction. It ...commonly presents with progressive dyspnea that is often associated with a history of pneumothorax or chylothorax particularly among females of reproductive age. In this report, we present a case of hydropneumothorax as the initial presentation of LAM in a 33-year-old woman, a rather rare presentation. We also discuss the pathogenetic mechanisms, the diagnosis, and treatment strategies using mTOR inhibitors like sirolimus.
Acute chest syndrome (ACS) is associated with prolonged hospitalization and high mortality in sickle cell population. The etiology of ACS is often multifactorial. It is well known that any chest pain ...that limits ventilation, blood flow or oxygenation establishes the risk of ACS. The independent contributory mechanism is fluid overload, resulting in pulmonary edema. In this report, we present a case of compound heterozygous Hb S/β+ thalassemia in a 32-year-old woman who presented with chest pain complicated by the development of pulmonary edema advancing to acute chest syndrome. We discuss the putative mechanisms might have led to pulmonary edema in this patient including hydration with underlying renal defect, along with a vicious cycle of vascular changes that resulted into pulmonary edema and consequently ACS.
Heparin induced thrombocytopenia (HIT) is serious disorder that occurs in a small percentage of patients following exposure to heparin. HIT can further be classified into two types: HIT type 1 and ...type 2. Type 2 HIT is a potentially life threatening with clinically significant outcomes. It presents with thrombocytopenia and evidence of thrombus formation in the presence of antibody formation. Additionally, several variations of HIT exist, including delayed onset HIT and refractory HIT, known collectively as autoimmune HIT (aHIT). Here we present a case of delayed onset and refractory HIT in a patient with little heparin exposure, discovered only after cardiac intervention for suspected STEMI. Significant thrombotic events occurred thereafter, including radial artery stenosis and intracardiac thrombus. Treatment with argatroban was ineffective. Significant resolution of thrombocytopenia was seen several weeks after infusion with IVIG, thus depicting further suspicion for refractory HIT. IVIG for aHIT treatment is traditionally chosen only if the disease process is refractory to other anticoagulation efforts due to the potential risk for increasing thrombotic risk with IVIG infusion. Our case illistrate the rare presentation of aHIT and the use if IVIG to successfully treat thrombocytopenia in refractory HIT.
Patients with cardiomyopathy (CM) are at increased risk for sudden cardiac death (SCD), specifically, secondary to ventricular arrhythmias such as ventricular tachycardia (VT) or ventricular ...fibrillation (VF). Those that have CM are further stratified based on risk of death from ventricular arrhythmias. If determined high risk, implantable cardioverter-defibrillators (ICD), anti-arrhythmic medication or even ablation procedures are considered in order to minimize the risk of SCD. Ablation procedures have increased in number over the years, along with the recognition of high-risk CM patients. Along with the increase in number of ablation procedures, complications from such procedures have decreased in number and the ventricular arrhythmia ablation remains a relatively low-risk procedure. Here we describe a patient with known CM, specifically hypertrophic cardiomyopathy (HCM) with a relatively rare and high-risk complication, being ventricular aneurysm. HCM patients with ventricular aneurysm are often referred for ablation procedures as they are at a significantly higher risk for SCD due to arrhythmias. Our patient not only underwent an ablation procedure, but suffered from the rare complication of tamponade, which occurs on average <2% annually. Although, risk assessments exist for stratifying CM as high-risk prior to invasive procedures, risk assessments are lacking for the specific population of HCM patients with LV aneurysm, thus presenting us with an area for further research.
Since the introduction of heparin as part of the management of acute coronary syndrome, the occurrence of pulmonary embolism (PE) as a complication of myocardial infarction (MI) have somewhat been ...unheard of. Given this rarity, its recognition is now a formidable challenge.
We present a case of a previously well 56-year-old male who presented with typical chest pain radiating to the left arm and dizziness. Initial vital signs revealed a blood pressure of 95/65 mmHg and his heart rate was 42 bpm. Physical exam revealed a middle-aged male in no acute distress with cool extremities, normal heart sounds, no murmurs and flat neck veins. His chest was clear to auscultation, abdomen benign and clinically, he was euvolemic. His first electrocardiogram (EKG) was significant for bradycardia with heart block and junctional escape rhythm and ST depressions in the anteroseptal leads. Subsequent right sided EKG showed sinus bradycardia with ST segment elevations in leads II, III and aVF. Troponin I was 0.95 ng/L normal <0.04 ng/L and initial transthoracic echo (TTE) was unremarkable with an ejection fraction (EF) of 55-60%. He was given loading doses of aspirin and clopidogrel, heparin drip initiated, and he proceeded to cardiac catheterization which revealed a 60
stenotic lesion of the mid LAD and a large filling defect with 100% stenosis of the mid RCA consistent with thrombus. Post procedure TTE revealed an EF of 40%, septal and posterior hypokinesis, right ventricular regional wall motion abnormality of the basal and mid free wall with apical hypercontractility (McConnell's sign) suggestive of PE. CT pulmonary angiography revealed bilateral pulmonary emboli and anticoagulation therapy was initiated.
This case illustrates that pulmonary embolism is a potential complication of RV infarction and its early identification is critical for implementation of anticoagulation therapy for this potentially fatal condition if left undiagnosed and untreated.
With the advent of hydroxyurea, the sickle cell population has been enjoying a prolonged life span as compared to the pre-hydroxyurea era. Traditionally, acute complications of sickle cell disease ...includes acute chest syndrome, MI and stroke. In this report we present a case of an elderly man with sickle cell disease who presented with intrahepatic cholestasis (SCIC); a rather rare and fatal complication of sickle cell hemoglobinopathy. The patient presented with jaundice and elevated bilirubin up to 53, his hospital course was complicated by coagulopathy and encephalopathy, and expired on day 43 of presentation after failing multiple therapeutic interventions including exchange transfusion. In this report, we will provide literature review and discuss the underlying pathophysiologic mechanisms of intrahepatic cholestasis in the sickle cell population highlighting the need for immediate recognition and institution of therapy for this fatal complication of sickle cell disease, particularly in elderly populations with low metabolic reserve.
COVID-19 caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is associated with significant cardiovascular dysfunction in patients with, and without, pre-existing ...cardiovascular disease 1. There are now well-documented cardiac complications of COVID-19 infection which include myocarditis, heart failure, and acute coronary syndrome 2. There is growing evidence showing that arrhythmias are also one of the major complications of COVID-19. We report a patient with no known cardiac conduction disease who presented with syncope, positive SARS-CoV-2 PCR, who was persistently bradycardic and subsequently developed sinus node dysfunction (SND). To date, there are a limited number of reports of sinus node dysfunction (SND) associated with COVID-19. We describe the clinical characteristics, potential pathophysiologic mechanisms and management of COVID-19 patients who experienced de novo SND.
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