Acquired hemophilia A and deep vein thrombosis attributable to the Pfizer-BioNTech SARS-CoV-2 mRNA vaccine-case report

Background: Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder that occurs in a sporadic, nonhereditary pattern. It is caused by circulating autoantibodies against clotting factor VIII that are triggered by several conditions. Moreover, AHA is clinically distinct from the inherited form of hemophilia A, with a different natural history and management approach, necessitating a high-index of suspicion in at-risk patients. Coronavirus disease 2019 (COVID-19) has emerged as a multisystemic disease whose manifestations are continuously being evaluated. There are few case reports of AHA associated with COVID-19 infection, while one case of AHA has been associated with COVID-19 vaccination. Similarly, deep venous thrombosis (DVT) frequently complicates COVID-19 infection, but two cases of DVT have been reported following COVID-19 vaccination. We report the occurrence of both AHA and DVT in a 63-year-old male patient within one week of receiving his first dose of the Pfizer-BioNTech SARS-CoV-2 mRNA vaccine. Case Description: Patient is a 63-year-old male who presented with a 3-day history of left lower extremity (LLE) swelling and pain. He was hemodynamically stable, but examination showed exquisite tenderness, ecchymosis, and pitting edema at the calf of the LLE. He had normal platelet counts at presentation but had mild anemia (11.9 g/dL) and elevated activated partial thromboplastin time (APTT) of 68.0 seconds. Venous Doppler ultrasound showed acute DVT in the left popliteal vein, necessitating commencement on heparin drip. He developed progressively worsening hematomas, symptomatic anemia that required red cell transfusions, and persistently elevated APTT despite stopping the heparin drip. Work up for pulmonary embolism, malignancy, and disseminated intravascular coagulopathy (DIC) were negative. Antiphospholipid antibodies and lupus anticoagulant were also negative. He had low factor VIII levels, tested positive for factor VIII inhibitor, and PTT mixing studies were consistent with acquired factor inhibitor. Treatment involved administration of Factor Eight Inhibitor Bypassing Activity (FEIBA) as well as intravenous methylprednisolone and cyclophosphamide. Following resolution of active bleeding with evidence of stable hemoglobin concentration, he was discharged home on oral prednisone and cyclophosphamide. Conclusion(s): This case report highlights the possibility of AHA and DVT as rare, potentially life-threatening adverse events that could occur following COVID-19 vaccination, which is currently the most effective tool employed in controlling the COVID-19 pandemic.Copyright © Annals of Blood. All rights reserved.

Deep Venous Thrombosis in Elderly Inpatients with Pneumonia

Background: The occurrence of deep vein thrombosis (DVT) in COVID-19 pneumonia has raised wide concern recently, but few studies have reported the incidence of DVT in other types of pneumonia. We evaluate the prevalence, risk factors and treatment of DVT in the elderly inpatients with pneumonia. Method(s): A cohort of 550 elderly inpatients (>= 75 years old) with pneumonia between 2017 and 2021 were reviewed. They were divided into DVT group and non-DVT groups on the basis of whether pneumonia was combined with new-found DVT. Clinical data were collected retrospectively. Patients with DVT were divided into anticoagulant group and non-anticoagulant groups on the basis of whether they received anticoagulant therapy. Result(s): Ninety-seven patients were included in the DVT group;453 in the non-DVT group. The incidence of DVT was 17.64%. Hospital stays were significantly longer for DVT patients than for non-DVT counterparts (p = 0.005). Coronary heart disease, heart failure, hyperlipidemia, bed rest, and elevated D-dimer were independent risk factors for DVT (p < 0.05). The rate of anticoagulant therapy in DVT group was 63.92% (62/97 cases). Follow-up showed that the continuous anticoagulant treatment rate was 48.39% (30/62 cases) at 3 months and 30.65% (19/62 cases) at 6 months. Conclusion(s): Elderly inpatients with pneumonia are at high risk of DVT. The combination of DVT and pneumonia may lead to prolonged hospitalization. Coronary heart disease, heart failure, hyperlipidemia, bed rest and elevated D-dimer are independent risk factors for DVT in these patients. The rate of regular anticoagulant treatment is low because of the high risk of bleeding.Copyright © 2022, Taiwan Society of Geriatric Emergency & Critical Care Medicine.

FOOD FOR THOUGHT: A CAUTIONARY TALE OF RIVAROXABAN FAILURE IN A MIDDLE-AGED MAN

SESSION TITLE: Rare Cases of Nervous System and Thrombotic Complication Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Rivaroxaban is a dose-dependent inhibitor of factor Xa. It is approved by the FDA to help reduce the risk of blood clots. Although bioavailability is not significantly affected at lower doses (80-100%), bioavailability at higher doses (≥15 mg) is as low as 66% when given without food [1] [3]. Here, we present a patient with poor oral intake who developed deep vein thrombosis (DVT) while on high dose Xarelto. CASE PRESENTATION: Our patient was a 48-year-old male with a history of pulmonary embolism diagnosed two months prior to admission (on 20 mg rivaroxaban daily at home) and morbid obesity who presented with dyspnea, fever, and decreased appetite. His viral PCR was positive for COVID-19, and CT angiogram showed multifocal ground glass opacities but was negative for pulmonary embolism. He was severely hypoxic on room air and required noninvasive ventilatory support in the intensive care unit. He was treated with remdesivir, dexamethasone, and baricitinib. His food intake was extremely poor due to near continuous use of noninvasive ventilation and decreased appetite. A nasogastric (NG) tube was offered, but the patient declined and elected to continue diminished oral feedings. He was able to take all of his home medications including rivaroxaban during this time. On day four, clinical nutrition was consulted because he had 3% loss of body weight. On day seven, the patient developed a fever of 101.6° F. Ultrasound of his lower extremities revealed acute DVTs in his left popliteal vein, posterior tibial vein, and peroneal vein. His anticoagulation was switched to full-dose enoxaparin and a NG tube was placed. On day ten, he was intubated due to worsening hypoxia. Unfortunately, the patient deteriorated into multiorgan failure and died on day seventeen. DISCUSSION: The latest expert guidelines suggest that direct oral anticoagulants (DOAC) should be used over vitamin K antagonists (VKA) in patients with acute venous thromboembolism (VTE) due to lower rates of major bleeding and recurrent VTE as well as convenience. Although VKAs are preferred in situations with extreme weight and renal impairment, DOACs have been proven to be effective for the large majority of patients [2]. Unlike rivaroxaban, the bioavailabilities of other DOACs like apixaban, edoxaban, and dabigatran are all unaffected by food and should be preferred in patients with tenuous oral intake [3]. It is well known that COVID-19 can produce a hypercoagulable state. This factor, combined with our patient's predisposition to blood clots and poor appetite, ended up precipitating new onset VTEs in his left leg despite rivaroxaban therapy. CONCLUSIONS: In patients with decreased oral intake, DOACs other than rivaroxaban should be considered. Patients should be briefed on the importance of taking high dose rivaroxaban with food. Our goal is to bring awareness to providers about this significant pharmacodynamic property of rivaroxaban. Reference #1: Mueck W, Stampfuss J, Kubitza D, Becka M. Clinical pharmacokinetic and pharmacodynamic profile of rivaroxaban. Clin Pharmacokinet. 2014 Jan;53(1):1-16. doi: 10.1007/s40262-013-0100-7. PMID: 23999929;PMCID: PMC3889701. Reference #2: Stevens SM, Woller SC, Baumann Kreuziger L, Bounameaux H, Doerschug K, Geersing GJ, Huisman MV, Kearon C, King CS, Knighton AJ, Lake E, Murin S, Vintch JRE, Wells PS, Moores LK. Executive Summary: Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest. 2021 Dec;160(6):2247-2259. doi: 10.1016/j.chest.2021.07.056. Epub 2021 Aug 2. PMID: 34352279. Reference #3: Lexicomp Online. Copyright © 1978-2022 Lexicomp, Inc. DISCLOSURES: No relevant relationships by Rishika Bajaj No relevant relationships by Ann Hylton No relevant relationships by Roger McSharry No relevant relationships by Krupa Solanki

A RARE CASE OF ASYMPTOMATIC COVID-19 INFECTION MASQUERADING AS PHLEGMASIA CERULEA DOLENS

SESSION TITLE: Look again: Infections and Mimics SESSION TYPE: Case Reports PRESENTED ON: 10/18/2022 11:15 am - 12:15 pm INTRODUCTION: Phlegmasia Cerulea Dolens (PCD) is a rare and critical condition caused by venous thrombosis requiring emergent treatment to prevent limb ischemia. COVID 19 has been widely reported to cause venous thromboembolism and compromise of tissue perfusion. We report a case of PCD in a patient with asymptomatic COVID-19 infection. CASE PRESENTATION: A 60 year-old female with no known medical history, unvaccinated for COVID-19 presented with sudden onset left lower extremity pain and swelling associated with numbness. Physical examination was remarkable for left lower extremity swelling with bluish discoloration, poikilothermia, and paraesthesia. Computed tomography angiogram (CTA) chest, abdomen and pelvis revealed left lower extremity deep vein thrombosis with compromised blood flow with focal thrombosis of the IVC extending inferiorly to the great saphenous and popliteal vein, along with small bilateral segmental and subsegmental pulmonary emboli. Diffuse Ground glass opacities suspicious for COVID-19 pneumonia. COVID-19 PCR was positive. Anticoagulation with heparin drip was initiated, and the patient underwent successful left iliocaval to popliteal vein thrombectomy and venoplasty by interventional radiology with successful restoration of circulation to the affected extremity. She was eventually transitioned to apixaban. She experienced marked improvement in her symptoms post procedure. DISCUSSION: Patients with COVID 19 develop venous thromboembolisms at an alarming rate despite thromboprophylaxis. The mechanism is likely explained by the virchow's triad (venous stasis, hypercoagulable state, vessel wall injury) in the setting of increased pro-inflammatory markers. We report the first case at our institution of PCD in the setting of COVID-19.We noted that our patient had a similar presentation as those reported in literature, which include acute leg swelling associated with pain and cyanosis. Complications include venous outflow obstruction, which can result in compartment syndrome with arterial ischemia, eventually progressing to gangrene of the affected limb. PCD is a very rare but life-threatening complication caused by extensive clot burden associated with acute limb ischemia and increased mortality rates. This condition requires emergent initiation of intravenous anticoagulation and thrombectomy with or without tissue plasminogen activator (tPA). If this condition is not treated in a timely fashion, it can result in acute limb ischemia and gangrene requiring amputation. CONCLUSIONS: Physicians should recognize PCD in patients who have been exposed to COVID-19 as it is a life-threatening condition which requires emergent initiation of anticoagulation and treatment. Diagnosis is usually made with clinical examination and ultrasonography or CT imaging. Management options include open thrombectomy with leg fasciotomy or catheter directed thrombolysis or percutaneous transluminal angioplasty. Reference #1: Chun TT, Jimenez JC, Pantoja JL, Moriarty JM, Freeman S. Phlegmasia cerulea dolens associated with acute coronavirus disease 2019 pneumonia despite supratherapeutic warfarin anticoagulation. J Vasc Surg Cases Innov Tech. 2020;6(4):653-656. doi:10.1016/j.jvscit.2020.10.002 Reference #2: Gutierrez JR, Volteas P, Skripochnik E, Tassiopoulos AK, Bannazadeh M. A Case of Phlegmasia Cerulea Dolens in a Patient With COVID-19, Effectively Ttreated With Fasciotomy and Mechanical Thrombectomy. Ann Vasc Surg. 2022 Feb;79:122-126. doi: 10.1016/j.avsg.2021.07.034. Epub 2021 Oct 10. PMID: 34644637;PMCID: PMC8502248 Reference #3: : Morales MH, Leigh CL, Simon EL. COVID-19 infection with extensive thrombosis: A case of phlegmasia cerulea dolens. Am J Emerg Med. 2020;38(9):1978.e1-1978.e3. doi:10.1016/j.ajem.2020.05.022 DISCLOSURES: No relevant relationships by Arij Azhar No relevant relationships by Louis Gerolemou No relevant relationships by Wael Kalaji No relevant relationships by Steven Miller N relevant relationships by jasparit minhas No relevant relationships by houman mirtorabi No relevant relationships by Kunal Nangrani No relevant relationships by Gaurav Parhar No relevant relationships by Kiran Zaman

Successful Treatment of Paraplegia Caused by Venous Hypertension Secondary to Extensive Iliocaval Deep Venous Thrombosis in the Absence of Phlegmasia Cerulea Dolens

Objectives: Paraplegia is known to complicate extensive iliocaval and lower extremity deep vein thrombosis (DVT) in rare instances. The most common pathophysiology is ischemia from severe venous hypertension in phlegmasia cerulea dolens. Less understood, however, is paresis or paraplegia in the absence of ischemia. We present a case of paraplegia in extensive iliocaval and lower extremity DVT without ischemia, which was successfully treated by percutaneous pharmacomechanical therapy. Methods: A 46-year-old African American woman with a history of hypertension, insulin-dependent diabetes mellitus, indwelling inferior vena cava filter since 2005, and recent coronavirus disease 2019 diagnosis, presented with acute abdominal pain with severe bilateral lower extremity edema, pain, and paresis. She was found to have bilateral iliocaval to tibial DVT (Fig 1). The patient was noted to have multiphasic arterial waveforms on ankle-brachial index and duplex ultrasound examination. Paresis quickly progressed to flaccid bilateral lower extremity paralysis. Neurologic workup was unrevealing. Despite her symptoms, thrombolytic therapy was delayed due to severe menstrual bleeding requiring a blood transfusion. Therapeutic anticoagulation was initiated. Results: On hospital day 10, the patient underwent 24-hour catheter-directed thrombolysis via bilateral popliteal vein access. Bilateral mechanical thrombectomy was then performed, achieving recanalization of the bilateral lower extremities, iliac veins, and inferior vena cava with minimal residual thrombus (Fig 2). The patient's edema and sensorimotor function immediately improved and never incurred lower extremity tissue ischemia. She was discharged on lifelong rivaroxaban. With physical therapy, the patient ambulated independently at 1 month postoperatively. Venous duplex ultrasound examination revealed continued iliocaval and lower extremity patency at 6 months postoperatively. Conclusions: We postulate that this patient suffered lower extremity paralysis secondary to cauda equina syndrome. Pharmacomechanical thrombectomy is a pragmatic means that reestablishes venous patency and relieves venous hypertension. This pathophysiology and its treatment should be considered in extensive iliocaval DVT and lower extremity neurologic compromise despite duration of paralysis. [Formula presented] [Formula presented]

Deep Vein Thrombosis, Septic Pulmonary Emboli and Methicillin-Resistant Staphylococcal Aureus Bacteremia Complicated by a Furuncle in a Child

Staphylococcal aureus infection in children is a major public health problem globally. It causes a varied spectrum of clinical disease including bacteremia, endocarditis, skin and soft tissue infection, pleuro-pulmaonry and osteo-articular infection. Deep vein thrombosis (DVT) is a known complication of staphylococcal infection. We report a case series which included, 10-year old boy developed DVT, septic pulmonary emboli and Methicillin-resistant Staphylococcal aureus (MRSA) bacteremia following a furuculosis and 13 year old girl with thrombosis of internal and external jugular vein, cavernous sinus with pulmonary emboli and MRA bacteremia. Both patients are previously healthy showed complete recovery after aggressive appropriate antibiotics, anticoagulants and supportive care. The high index of suspicion of DVT in MRSA infection is needed, prompt diagnosis and aggressive appropriate therapies improve the outcomes and minimize the complications.

How did we manage long COVID with CAPA (COVID Associated Pulmonary Aspergillosis) and DVT: A case report

Introduction: The invasion of lung tissue by a commensal like aspergillus after severe viral infection has been known.[1] But coronavirus has multiple fangs like immune dysfunction, precipitates new onset diabetes and hypercoagulability.[2] We hereby present a case of long COVID with pulmonary aspergillosis and deep vein thrombosis (DVT). Case Study: A 46 year old male presented with one and half month history of fever, cough with expectoration, hemoptysis. He had tested positive for COVID and diabetes two months before and had required intensive care treatment. The Chest Xray [Figure 1a] showed left upper lobe opacity. Chest CT Scan [Figure 2] showed cavity with central hypodense component within anterior segment of left upper lobe (bird's nest appearance). BAL from that segment grew aspergillus. The patient was started on voriconazole, hemoptysis was controlled and subsequently discharged. But 15 days later, he was readmitted with hemoptysis and left side calf pain and swelling. Lower limb venous doppler showed thrombosis of parts of Left Superficial femoral vein and popliteal vein. CTPA (CT Pulmonary Angiography) didn't show any filling defect. Patient was started on anticoagulation. After 8 weeks patient improved with significant clearing of lung lesion on chest X Ray [Figure 1b]. Discussion: Studies from Wuhan, China, reported secondary fungal infections in 35.3% critically ill patients.[3] Our patient, apart from other risk factors had received corticosteroids and the dose was more than 0.3 mg/kg/d and the duration was upto 4 weeks.[4] Conclusion: Post COVID cases with hemoptysis should be investigated properly considering the multiple pathogenic pathways that are implicated by this virus.

A case of dvt and pe and cardiac arrest in a patient with factor v leiden mutation and covid

Introduction Heterozygous Factor V Leiden (FVL) mutation is the most common inherited thrombophilia, most common in people of Northern European descent and in some Middle Eastern population. It increases risk of developing a deep venous thrombosis (DVT) by 5-to 7-fold, and is considered a weak risk factor, and most people never develop blood clots. Case report 19-year-old woman presented with left lower quadrant abdominal pain, left groin pain, chest tightness and shortness of breath that had started 1 week prior to presentation. Patient had tested positive for COVID-19 six months but had only minor symptoms and recovered without needing treatment. She had completed 2nd dose of Moderna Covid vaccine two weeks ago and had been using oral contraceptive pills (OCP) for two years. Family history was significant for a paternal uncle with history of blood clots. Physical exam revealed swelling and erythema in left lower extremity up to groin area. Doppler ultrasound showed an acute DVT in the left external iliac vein, common femoral vein, deep femoral vein, superficial femoral vein, and proximal popliteal vein. Spiral computed tomography imaging of chest showed pulmonary emboli (PE) in the segmental branches of the pulmonary arteries with mild dilation of the right ventricle. Cardiac echogram (TTE) showed intact right ventricular function. Patient underwent mechanical aspiration thrombectomy with extirpation of DVT, but the procedure was complicated by PEA arrest requiring CPR for about 50-60 mins. During episode, bedside TTE showed evidence of right ventricular dilation and RV strain concerning for a massive PE. She was cannulated for V-A ECMO then underwent therapeutic hypothermia, and was successfully decannulated after six days. Patient eventually recovered and was neurologically intact. She was discharged home on warfarin and aspirin. Hypercoagulable work-up was remarkable for heterozygous FVL mutation R506Q. Both her father and mother were found to have heterozygous FVL mutation and her sister was found to have homozygous FVL mutation. Discussion People with FVL have additional risk factors that contribute to the development of DVT, and FVL alone does not increase the risk of developing arterial thrombosis, heart attacks and strokes. DVTs in heterozygous FVL population are considered provoked DVTs requiring anti-coagulation for a definite period, like that in general population. Curiously, patients with severe COVID-19 infection requiring ventilator support were found to have factor V levels high above the normal reference range and were found to have elevated risk for blood clots. Use of OCPs, and perhaps a recent COVID infection, although mostly asymptomatic, might have contributed to hypercoagulability in our patient. FVL mutation is not considered a contraindication to having COVID vaccination and patients with hereditary clotting disorders are recommended to have the vaccine.

Catheter-Directed Thrombolysis of Iliocaval Thrombosis in Patients With COVID-19 Infection.

We present the characteristics and outcomes of the first 2 cases of catheter-directed thrombolysis performed in patients presenting with coronavirus disease-2019 (COVID-19)-related iliocaval thrombosis. (Level of Difficulty: Beginner.).