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Autoimmune cytopenias associated with autoantibodies to nuclear envelope polypeptides

Abstract : A subset of anti-nuclear autoantibodies (ANA) are directed against nuclear envelope (NE) polypeptides and display by indirect immunofluorescence (IIF) a ring-like fluorescent pattern. We report herein 19 patients with autoimmune cytopenias associated with antibodies (Abs) to NE polypeptides. Anti-NE specificity was determined by immunoblot, using NE preparations and purified lamina fractions. Eleven sera reacted with lamin B(1), and two reacted with both lamin B(1) and an unidentified 150-kDa protein (p150). One serum reacted with only p150. Four sera reacted with lamins A and C, and one reacted with and an unidentified 52-kDa NE polypeptide (p52). Autoimmune cytopenias included hemolytic anemia (7 cases), thrombocytopenia (13 cases), and neutropenia (6 cases). Five patients had 2 (3 cases) or 3 (2 cases) different cytopenias. Antiphospholipid antibodies (APLA) were detected in 14 patients, 2 of whom experienced thromboembolic events. A liver disorder was present in 7 patients. Systemic lupus erythematosus and lupus-like syndrome were diagnosed in 11 and 2 patients, respectively. Cytopenias responded to steroids alone (13 patients), or together with intravenous immunoglobulins (2 patients), or cyclophosphamide (2 patients). Two patients did not require treatment. Our results suggest that anti-NE Abs need to be sought for in patients with peripheral cytopenias, particularly when they are associated with APLA and/or liver disorders. Their detection strongly suggests an autoimmune process. Such cytopenias are often manifestations of a lupus or lupus-like disease and are responsive to steroids.
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Contributor : Valérie Gouilleux Connect in order to contact the contributor
Submitted on : Monday, December 30, 2019 - 12:14:51 PM
Last modification on : Tuesday, September 13, 2022 - 12:46:07 PM

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Paul Coppo, Jean-Pierre Clauvel, Djaouida Bengoufa, Vincent Fuentes, Valérie Gouilleux-Gruart, et al.. Autoimmune cytopenias associated with autoantibodies to nuclear envelope polypeptides. American Journal of Hematology, Wiley, 2004, 77 (3), pp.241-249. ⟨10.1002/ajh.20188⟩. ⟨hal-02425326⟩



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