Therapeutic plasma exchange for anticoagulant-refractory antiphospholipid syndrome with severe ischemic and necrotic skin lesions: A case series

• Published in: Transfusion and apheresis science 2021-10, Vol.60 (5), p.103192-103192, Article 103192
• Main Authors: Croles, F. Nanne, te Boekhorst, Peter A.W., Leebeek, Frank W.G., Jansen, A.J. Gerard
• Format: Article
• Language: English
• Subjects: Aged; Antibodies, Anticardiolipin - blood; Anticoagulant-refractory; Anticoagulants - chemistry; Antiphospholipid syndrome; Antiphospholipid Syndrome - immunology; Antiphospholipid Syndrome - pathology; Antiphospholipid Syndrome - therapy; APS; beta 2-Glycoprotein I - immunology; Female; Glucocorticoids - therapeutic use; Heparin, Low-Molecular-Weight; Humans; Immunoglobulin G - immunology; Immunoglobulin M - immunology; Ischemia - pathology; Ischemia - therapy; Lupus Coagulation Inhibitor - immunology; Male; Middle Aged; Necrosis - pathology; Necrosis - therapy; Plasma Exchange - methods; Skin Diseases - pathology; Skin Diseases - therapy; Therapeutic plasma exchange; Thrombosis; Thrombosis - immunology
• ISSN: 1473-0502; 1878-1683
• DOI: 10.1016/j.transci.2021.103192

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by clinical findings including thrombosis and/or obstetric complication and laboratory findings, e.g. ≥1 positive antiphospholipid antibodies (aPL) (lupus anticoagulant, anticardiolipin IgG/IgM and/or anti-β2-glycoprotein IgG/IgM). A rare APS clinical entity is severe necrosis which is difficult to treat and often does not respond to anticoagulant therapy. Three consecutive patients with primary or secondary APS who presented with necrotic skin lesions secondary to APS were treated with therapeutic plasma exchange (TPE), glucocorticoids and low-molecular-weight heparin. All patients had a rapid-onset, either full or significant recovery of their APS-related necrotic lesions. Upon treatment, one patients showed resolution of lupus anticoagulant. Two patients had a decrease of at least 88 % in aPL titers after the initial treatment, and were kept on TPE maintenance every 5–6 weeks. None of the patients experienced significant side effects of the TPE. This is the first case series showing the clinical benefits of TPE in patients with ischemic and necrotic skin lesions due to severe anticoagulant-refractory vascular APS.