A CASE OF PHLEGMASIA CERULEA DOLENS AND MAY-THURNER SYNDROME IN A YOUNG MALE WITH TRIPLE-POSITIVE ANTIPHOSPHOLIPID ANTIBODY SYNDROME

Abstract

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Phlegmasia cerulea dolens (PCD) is an uncommon form of deep vein thrombosis (DVT) typically presenting with acute limb swelling, pain, and cyanosis. Diagnosis of PCD should be made promptly as these patients may develop limb-threatening complications such as gangrene and compartment syndrome. We describe a young male presenting with his first episode of provoked venous thromboembolism (VTE) as PCD complicated with May-Thurner syndrome (MTS) and Antiphospholipid antibody syndrome (APS). CASE PRESENTATION: A 37-year-old obese male with type 2 diabetes, hypertension, and hyperlipidemia was directly admitted to the medicine floor with extensive left lower extremity DVT (including ileofemoral vein thrombosis) found on outpatient duplex ultrasonography. At initial hospitalization, he was tachycardic but without respiratory distress or hemodynamic instability. His left lower extremity was tender and edematous with cyanosis. Diagnosis of PCD was made and patient was treated with subcutaneous enoxaparin. He received catheter-directed thrombolysis for PCD with alteplase and heparin infusion. During left leg venogram, he was discovered to have MTS, and subsequently underwent stent placement of left common iliac vein, external iliac vein, and common femoral vein. After stent placement, patient received bridging to warfarin with enoxaparin and was discharged home two days later. Patient’s family history was significant for Systemic Lupus Erythematosus. During hospitalization, his antibody testing revealed positivity for anti-cardiolipin, anti-beta-2 glycoprotein antibodies in addition to positive functional lupus anticoagulant assay. Positivity confirmed with repeat testing of anti-cardiolipin antibodies and lupus anticoagulant assay at 15 weeks after hospitalization - confirming the diagnosis of triple-positive APS. DISCUSSION: Treatment of PCD is anticoagulation therapy with adjunctive catheter-directed thrombolysis. Outcomes comparing additional catheter-directed thrombolysis versus anticoagulation alone have shown that combination treatment reduces the incidence of post-thrombotic syndrome in patients with acute iliofemoral DVT. Recognition of DVT associated with MTS is important as treatment includes stent placement to maintain venous outflow. Recognition of triple-positive APS is an indication for extended anticoagulation as this portends a high risk of recurrent VTE. Patient was discharged with warfarin therapy because the use of direct oral anticoagulation in patients with APS remains controversial. Recent studies showed increased risk of recurrent thrombosis, particularly in patients with APS who are triple-positive. CONCLUSIONS: We reported a case, 37 year old male with phlegmasia cerulea dolens and May-Thurner syndrome resulting from triple-positive antiphospholipid antibody syndrome. Reference #1: Casey ET, Murad MH, Zumaeta-garcia M, et al. Treatment of acute iliofemoral deep vein thrombosis. J Vasc Surg. 2012;55(5):1463-73 Reference #2: Haig Y, Enden T, Grøtta O, et al. Post-thrombotic syndrome after catheter-directed thrombolysis for deep vein thrombosis (CaVenT): 5-year follow-up results of an open-label, randomised controlled trial. Lancet Haematol. 2016;3(2):e64-71 Reference #3: Dufrost V, Risse J, Reshetnyak T, et al. Increased risk of thrombosis in antiphospholipid syndrome patients treated with direct oral anticoagulants. Results from an international patient-level data meta-analysis. Autoimmun Rev. 2018;17(10):1011-1021 DISCLOSURES: No relevant relationships by Waqas Adeel, source=Web Response No relevant relationships by Robert Decker, source=Admin input No relevant relationships by Shuisen Li, source=Web Response No relevant relationships by Preysi Patel, source=Web Response No relevant relationships by Chun Siu, source=Web Response