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Autoimmune cytopenias associated with autoantibodies to nuclear envelope polypeptides
Author(s) -
Coppo Paul,
Clauvel JeanPierre,
Bengoufa Djaouida,
Fuentes Vincent,
GouilleuxGruart Valérie,
Courvalin JeanClaude,
Lassoued Kaiss
Publication year - 2004
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20188
Subject(s) - autoantibody , medicine , immunology , antibody , autoimmune hemolytic anemia , systemic lupus erythematosus , autoimmune disease , connective tissue disease , antiphospholipid syndrome , disease
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. Am. J. Hematol. 77:241–249, 2004. © 2004 Wiley‐Liss, Inc.