Relapsing Evans syndrome and systemic lupus erythematosus with antiphospholipid syndrome treated with Bortezomib in combination with plasma exchange

Relapsing Evans syndrome (ES) and systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome (APS) is very rare association. Coexistence of these syndromes is potentially fatal and require high-dose combined immunosuppressive therapy. We describe a case of successful use of Bortezom...

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Bibliographic Details
Published in:Clinical immunology (Orlando, Fla.) Fla.), 2019-02, Vol.199, p.44-46
Main Authors: Tkachenko, Olga, Lapin, Sergey, Maslyansky, Alexey, Myachikova, Valentina, Mikhailova, Liya, Gilburd, Boris
Format: Article
Language:English
Subjects:
Adult
Anemia, Hemolytic, Autoimmune - immunology
Anemia, Hemolytic, Autoimmune - therapy
Antiphospholipid syndrome
Antiphospholipid Syndrome - immunology
Antiphospholipid Syndrome - therapy
beta 2-Glycoprotein I - immunology
Bortezomib
Bortezomib - therapeutic use
Evans syndrome
Hemolysis
Humans
Lupus Erythematosus, Systemic - immunology
Lupus Erythematosus, Systemic - therapy
Male
Plasma Exchange
Recurrence
Thrombocytopenia - immunology
Thrombocytopenia - therapy
Thrombosis
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Summary:Relapsing Evans syndrome (ES) and systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome (APS) is very rare association. Coexistence of these syndromes is potentially fatal and require high-dose combined immunosuppressive therapy. We describe a case of successful use of Bortezomib and plasma exchange in a patient with ES and APS refractory to standard therapy. Thirty-two-year-old male who presented episodes of relapsing hemolytic anemia, pancytopenia and multiple thrombosis with positive direct and indirect antiglobulin test result, lupus anticoagulant and medium titer of anti-beta-2-glycoprotein 1 and anti-cardiolipin antibodies was diagnosed with ES and SLE with secondary APS. High-dose therapy by steroids and Cyclosporin A were started with temporary improvement. There was also no stable improvement with Rituximab and Cyclophosphamide. Bortezomib in combination with cyclosporine A and plasma exchange was introduced. He had stable improvement in hematological parameters with no evidence of relapse of hemolytic crisis or thrombosis during a follow-up for 1 year.
ISSN:1521-6616
1521-7035
DOI:10.1016/j.clim.2018.12.010