Trends of Ordering Hypercoagulability Work-Up at an Academic Medical Center.

BACKGROUND: Venous thromboembolism is a significant clinical event, with an annual incidence of 1-2 per 1000 population. Risk factors include recent surgery, prolonged immobility, oral contraceptive use, and active cancer. Inherited risks include protein C and S deficiencies, antithrombin deficiency, factor V Leiden mutation and prothrombin. These factors can be tested to guide therapy, but current evidence suggests that testing for inherited thrombophilia is not recommended in most inpatient settings. In the era of high value care, hypercoagulable testing for VTE creates a financial burden for the hospital and patients. We performed a retrospective chart review of hypercoagulable orders on VTE patients at our institution. METHODS: Institutional Review Board approval was obtained. A total of 287 adult patients admitted over a 3-month period with the diagnosis of VTE were included. Patients were identified via ICD-10 codes and data were collected from electronic medical records. Patient characteristics, provoked versus unprovoked VTE, and relative contraindications for hypercoagulability work-up were analyzed. Our primary outcome was to assess the appropriateness of thrombophilia testing in VTE patients based on screening guidelines. Our secondary outcome was to analyze the cost burden of ordering these tests. RESULTS: A total of 287 patients were included in our data analysis. Patient risk factors for VTE were malignancy, previous DVT, immobilization, surgery 3 months prior, and central line placement. Fifty-seven of 287 patients had at least one hypercoagulable test ordered during hospitalization which did not adhere to guidelines. Misuse of testing occurred during active thrombosis, active anticoagulation, presence of risk factors, first episode of VTE, and malignancy. The cost of ordering these 5 thrombophilia tests totaled over $40,000. CONCLUSION: In our study, numerous patients were tested without compliance to standard recommendations, which created financial and value-based burdens on our health care system. Increased awareness among clinicians is thus warranted to ensure high value care.

Post-traumatic thrombotic microangiopathy: What trauma surgeons need to know?

Thrombotic microangiopathy (TMA) is characterized by systemic microvascular thrombosis, target organ injury, anemia and thrombocytopenia. Thrombotic thrombocytopenic purpura, atypical hemolytic uremic syndrome and Shiga toxin E-coli-related hemolytic uremic syndrome are the three common forms of TMAs. Traditionally, TMA is encountered during pregnancy/postpartum period, malignant hypertension, systemic infections, malignancies, autoimmune disorders, etc. Recently, the patients presenting with trauma have been reported to suffer from TMA. TMA carries a high morbidity and mortality, and demands a prompt recognition and early intervention to limit the target organ injury. Because trauma surgeons are the first line of defense for patients presenting with trauma, the prompt recognition of TMA for these experts is critically important. Early treatment of post-traumatic TMA can help improve the patient outcomes, if the diagnosis is made early. The treatment of TMA is also different from acute blood loss anemia namely in that plasmapheresis is recommended rather than platelet transfusion. This article familiarizes trauma surgeons with TMA encountered in the context of trauma. Besides, it provides a simplified approach to establishing the diagnosis of TMA. Because trauma patients can require multiple transfusions, the development of disseminated intravascular coagulation must be considered. Therefore, the article also provides different features of disseminated intravascular coagulation and TMA. Finally, the article suggests practical points that can be readily applied to the management of these patients.

Diagnostic Enigma: Spindle Cell Sarcoma of the Aorta Presenting as Pulmonary Embolism and Chronic Anaemia.

Primary aortic sarcoma is a rare and aggressive malignancy with only approximately 190 cases reported in the literature. While angiosarcoma and intimal sarcomas represent an estimated 67.7% of malignant aortic tumours, spindle cell sarcomas are even more exclusive, consisting of only 0.9% of malignant aortic tumours. Differentiated from other malignant aortic tumours, spindle cell sarcomas are of mesenchymal origin and usually express vimentin and osteopontin. Clinical presentations are variable and nonspecific, ranging from back pain, abdominal pain or elevated blood pressure, misleading to differentials like pulmonary emboli or aortic aneurysms such as in our case here. In this article, we discuss the finding of an extremely rare aortic sarcoma masquerading as a pulmonary embolism. The patient underwent surgical resection; however, the course was complicated by the development of brain metastases and intracranial haemorrhage. The literature is expanding regarding the evolution of adjuvant chemotherapy and radiation therapy in the treatment of these patients. The exact pathogenesis of spindle cell sarcomas is unknown but thought to be related to the MDM2-p53 pathway. The development of spindle cell sarcomas may be related to Li-Fraumeni syndrome, which should be on the differential for these patients. This case highlights the importance of identifying aortic sarcomas in patients who present with signs and symptoms of peripheral embolization as the diagnosis can be easily misconstrued for thrombus or aortic aneurysm, leading to a delay in proper and timely management. We herein emphasize that aortic sarcomas should be included in the clinician's working differential due to the poor prognosis and outcomes that these aggressive tumours carry. LEARNING POINTS: Malignant aortic tumours are rare and can present with a multitude of symptoms ranging from constitutional symptoms to abdominal discomfort to unexplained hypertension. Spindle cell sarcomas represent 1 of the least common malignant aortic tumours reported in the literature.Malignant aortic tumours have a poor prognosis, and of the various types of malignant aortic tumours, aortic sarcomas have a particularly poor prognosis with a 5-year survival rate of 8%.The exact pathophysiology of these malignancies is unknown but is thought to be related to the MDM2-p53 pathway and may be related to Li-Fraumeni syndrome.

Propylthiouracil-Induced Anti-Neutrophil Cytoplasmic Antibody Vasculitis Presenting with Red Eye Followed by Pulmonary Hemorrhage: Diagnostic and Management Considerations.

BACKGROUND Drug-induced anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) should be suspected in patients on certain medications who present with inflammatory ocular, constitutional, pulmonary, and/or renal manifestations. Here, we present a case of propylthiouracil (PTU)-induced AAV presenting initially with red eye, and review important diagnostic and management considerations for this uncommon disorder. CASE REPORT A 34-year-old woman with hyperthyroidism taking PTU presented with red eye, later followed by fevers and hemoptysis. She was found to have episcleritis, diffuse alveolar hemorrhage, and microhematuria. The infectious diseases workup was unrevealing. Laboratory evaluations were notable for a high-titer perinuclear ANCA and elevated anti-myeloperoxidase antibodies. Renal function was normal. She was ultimately diagnosed with PTU-induced AAV. PTU was promptly discontinued and she was treated with pulse-dose methylprednisolone for 3 days, followed by prednisone 60 mg daily. A kidney biopsy revealed pauci-immune focal segmental necrotizing and crescentic glomerulonephritis. Given an allergy to methimazole, she underwent thyroidectomy and was ultimately treated with rituximab. Her steroid doses are progressively being tapered and she has complete resolution of symptoms. CONCLUSIONS PTU-induced AAV is a rare and serious condition. Our patient presented with ocular symptoms prior to more commonly recognized pulmonary and renal manifestations. Patients may have favorable outcomes if PTU is discontinued promptly, but patients with vital-organ involvement may require treatment with steroids and may need additional immunosuppression.

Iatrogenic Cushing's Syndrome Following Intra-Articular Triamcinolone Injection in an HIV-Infected Patient on Cobicistat Presenting as a Pulmonary Embolism: Case Report and Literature Review.

BACKGROUND: Iatrogenic Cushing's syndrome (ICS) typically develops after long-term exposure to corticosteroids, but it can occur after a single dose in patients treated with cobicistat or ritonavir for HIV. We present a patient who developed ICS due to the interaction between cobicistat and triamcinolone, a review of the literature, and what to our knowledge is the first case of ICS presenting as a pulmonary embolism. CASE PRESENTATION: A 55-year old male with a past medical history of human immunodeficiency virus, undetectable for 15 years and currently on elvitegravir/cobicistat/emtricitabine/tenofovir alafenamide, received 2 intra-articular injections of triamcinolone one month apart for a Baker's cyst in his right knee. He used nasal fluticasone for 9 days in-between the injections. After his second knee injection, he developed easy bruising and friable skin. Over the coming months, he experienced weight gain and Cushingoid facies. Four months after the knee injections he developed a pulmonary embolism and deep vein thrombosis treated with warfarin. The Cushingoid facies prompted an evaluation and diagnosis of ICS along with hypothalamic pituitary adrenal axis suppression. CONCLUSION: This case demonstrates the need to monitor patients on pharmacological boosters with any exposure to corticosteroids, whether it be injected, inhaled, topical, oral or intravenous, as it can lead to profound adrenal suppression and ICS.

Acute Fulminant Hepatic Failure and Renal Failure Induced by Oral Amiodarone: A Case Report and Literature Review.

Amiodarone is a class III antiarrhythmic agent that inhibits adrenergic stimulation by blocking alpha and beta receptors. It prolongs action potential and refractory period in myocardial tissue. Its remarkably long half-life is associated with a myriad of adverse events. Here, we present an 85-year-old male patient who was started on amiodarone for atrial flutter. After three oral doses, he developed fulminant hepatic failure and acute renal failure, which resolved after stopping amiodarone. While fulminant hepatic failure is rare, it has been seen in less than 2% of patients. Alternative theories behind susceptibility to amiodarone-induced hepatic injury and acute kidney injury are discussed here.

Chronic Kidney Disease in Patients Undergoing Cardiac Device Placement: Results of a Retrospective Study.

BACKGROUND: Cardiovascular issues (especially arrhythmia and sudden cardiac death) are one of the most common causes of mortality in patients with chronic kidney disease (CKD). To minimize cardiac mortality, these patients frequently require various cardiac devices, such as pacemakers, loop recorders, and defibrillators which can compromise their vascular access. In this study, we aim to determine the prevalence of CKD in patients undergoing cardiac device placement and their progression of CKD. METHODS: Institutional review board approval was obtained for this study. A total of 688 patients undergoing cardiac device placement were included in this study over a 3-year period at Jersey Shore University Medical Center. Demographic characteristics, comorbidities, base-line renal functions during the procedure, types of cardiac devices, sites of vascular access and follow-up renal function when available were assessed retrospectively. Patients were categorized into CKD stages 1 - 5 based on the National Kidney Foundation-Kidney Disease Outcomes Quality Initiative (NKF-KDOQI) guidelines. The patients who were already on hemodialysis were excluded in this study. RESULTS: The average age of the patient were 73.9 years with male predominance (60%). A total of 227 patients (33%) had estimated glomerular filtration rate (eGFR) < 60 mL/min consistent with the evidence of advanced-stage CKD (stages 3 - 5) at the time of cardiac device placement. The most common types of device placements were new insertion/replacement of atrial and ventricular leads (39.5%), loop recorder implantation (21.1%) and generator changes on an already implanted device (11%). Only 4% (28/688) had a leadless cardiac device placement. The most common access sites were subclavian (47.1%), axillary (32.3%) and femoral (12.2%). CONCLUSIONS: The present study demonstrated that nearly one-third of the patient undergoing cardiac device placement had an advanced degree of renal failure. Because CKD is a progressive disease, many of these patients might require renal replacement therapy in the future. Transvenous devices is not a good choice in this group of patients as they will ultimately require an arteriovenous fistula. Subcutaneous leadless cardiac device insertion might be a better option in patients with advanced CKD.

A Unique Presentation of Spontaneous Compartment Syndrome due to Acquired Hemophilia A and Associated Malignancy: Case Report and Literature Review.

Hemophilia is a bleeding diathesis that is most commonly congenital and causes a tendency for significant bleeding during procedures and often manifests as hemarthrosis. However, more rarely, hemophilia can be acquired. Our paper focuses on acquired hemophilia A (AHA), which is caused by the development of an autoantibody (an inhibitor) to factor VIII. A 61-year-old man with a past medical history of type II diabetes mellitus, hypertension, hyperlipidemia, hypothyroidism, and obstructive sleep apnea presented to the emergency department with severe right lower extremity pain and swelling of 2-day duration. He was found to have compartment syndrome and underwent emergent fasciotomy of his right leg. After surgery he still had significant bleeding, despite transfusions and administration of fresh frozen plasma (FFP) by the surgical team. He was later diagnosed with AHA, but was not adequately responsive to factor VII, factor VIII, steroids nor rituxan and unfortunately had his right lower extremity amputated. He had a prolonged hospital course, which included Streptococcus bovis bacteremia and a code stroke for which head computed tomography (CT) showed probable metastasis. It was acknowledged he had probable metastatic colon cancer, which was not confirmed as the patient transitioned to hospice care. Rather than hemarthrosis, patients with AHA tend to have bleeding in soft tissue or the gastrointestinal tract. AHA can have underlying causes, such as malignancy. AHA associated with malignancy is associated with poorer outcomes and tends to improve with treatment of the underlying malignancy. Therefore, it is important to quickly identify these patients and screen them for underlying etiologies.

Rivaroxaban Related Bilateral Adrenal Hemorrhage: A Rare Complications of Direct Oral Anticoagulants - A Case Reports.

BACKGROUND Adrenal hemorrhage is an uncommon and under-recognized disorder with a wide array of etiologies ranging from pregnancy to septic shock. It is one of the complications of anticoagulation therapy, including direct anticoagulant medications. CASE REPORT Here, we present a case of a 68-year-old female with recent right knee arthroplasty who was on rivaroxaban for deep vein thrombosis (DVT) prophylaxis presented to the emergency department (ED) for severe acute onset abdominal pain, computed tomography (CT) of abdomen and pelvis revealed possible left adrenal hemorrhage that was confirmed with magnetic resonance imaging (MRI). On repeat CT, her unilateral adrenal hemorrhage converted to a bilateral adrenal hemorrhage (BAH) and, as a result, the patient developed adrenal insufficiency. CONCLUSIONS An undiagnosed and untreated adrenal hemorrhage can have catastrophic consequences, leading to adrenal insufficiency with potential shock and death. Therefore, it is important for clinicians to have an increased awareness and knowledge about adrenal hemorrhage.