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Acute Liver Necrosis in a Sars-Cov-2 Positive Patient with Triple Positive Antiphospholipid Syndrome

V. La Mura1,2, R. Gualtierotti1,3, I. Martinelli1, B. Ferrari1, A.M. Ierardi4, N. Bitto1, G. Ghigliazza1, A. Giachi1, M. Maggioni5, M. Cugno1,3, A.L. Fracanzani3,6, G. Carrafiello4,7, F. Peyvandi1,3

1IRCCS Fondazione Ca' Granda - Ospedale Maggiore Policlinico - UOC Medicina Generale Emostasi e Trombosi, Milan, Italy, 2Università degli Studi di Milano - Dipartimento di Scienze Biomediche per la Salute, MIlan, Italy, 3Università degli Studi di Milano - Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Milan, Italy, 4IRCCS Fondazione Ca' Granda - Ospedale Maggiore Policlinico – UO Radiologia, Milan, Italy, 5IRCCS Fondazione Ca' Granda - Ospedale Maggiore Policlinico - UO Anatomia Patologica, Milan, Italy, 6IRCCS Fondazione Ca' Granda - Ospedale Maggiore Policlinico - UOC Medicina Generale a Indirizzo Metabolico, Milan, Italy, 7Università degli Studi di Milano - Dipartimento di Scienze della Salute, Milan, Italy

Abstract Number: PB0299

Meeting: ISTH 2021 Congress

Theme: COVID and Coagulation » COVID and Coagulation, Clinical

Background: Several thrombotic manifestations have been reported with SARS-Cov-2 infection including liver vascular involvement.

Aims: We present a dramatic case of acute liver necrosis in a 36-year-old SARS-Cov-2 positive Italian woman with no respiratory symptoms and triple positive antiphospholipid syndrome (APS).

Methods: The patient was referred to our University Hospital for acute hypertransaminasemia and liver failure (Figure). She had systemic lupus erythematosus (positivity for: ANA, anti-dsDNA, complement activation, Coombs; thrombocytopenia, previous arthritis). Anti-phopspholipid antibodies (aPL) were detected for the first time in 2015 during routine pregnancy screening and chronically treated with aspirin. Apparently, no venous/arterial nor obstetric events were recorded up to the recent hospitalization.

Results: At arrival, US-Doppler and CT-scan were consistent with signs of chronic liver disease and occlusion of the three hepatic veins defining a Budd-Chiari syndrome. We opted for a stepwise approach considering anticoagulation (clexane 100UI/Kg b.i.d) the first line of therapy before any invasive intervention. Dexamethasone 6 mg/day b.i.d., 6 sessions of plasma-exchange, i.v.-immunoglobulin were sequentially planned to revert the liver damage sustained by aPL. After 5-days, two hepatic-veins resulted recanalized in association with amelioration of liver-enzyme/function and aPL quantification. Then we performed hepatic vein catheterization and transjugular liver biopsy. The histology showed multiple areas of necrosis associated with liver cirrhosis. Unexpectedly, no signs of acute Budd-Chiari were observed (e.g. intraparenchymal hemorrhages, centrilobular congestion, sinusoidal dilation). Other etiologies were also excluded and we hypothesized the involvement of small arteries of the liver in a triple positive APS in a patients with SLE. We finally addressed the patient to a liver transplant program and a tight multispecialistic follow-up.

Conclusions: Thrombosis of arterial/venous vessels or microcirculation causes liver damage in some patients with aPL. Our report suggests that SARS-Cov-2 can exacerbate this prothrombotic condition determining a life-threatening complication such as acute liver failure.

JPG; Figure

To cite this abstract in AMA style:

La Mura V, Gualtierotti R, Martinelli I, Ferrari B, Ierardi AM, Bitto N, Ghigliazza G, Giachi A, Maggioni M, Cugno M, Fracanzani AL, Carrafiello G, Peyvandi F. Acute Liver Necrosis in a Sars-Cov-2 Positive Patient with Triple Positive Antiphospholipid Syndrome [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/acute-liver-necrosis-in-a-sars-cov-2-positive-patient-with-triple-positive-antiphospholipid-syndrome/. Accessed August 16, 2022.

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