Pure red cell aplasia in a Sjögren's syndrome/lupus erythematosus overlap patient.
Hematological complications of systemic lupus erythematosus usually include anemia of chronic disease and peripheral destruction of blood cells. We describe the case of a young woman with Sjögren's syndrome'lupus erythematosus overlap, mother of infant with congenital heart block, complicated by pure red cell aplasia. The patient was asymptomatic until the onset of severe anemia. A serum inhibitor of erythropoiesis was detected before the onset of immunosuppression. Bone marrow examination showed a low CD4:CD8 ratio, an immune defect possibly linked with the unrestrained production of antibodies against erythroid progenitor cells.
Mavragani
Clio P
CP
Department of Pathophysiology, School of Medicine, National University of Athens, Greece.
Vlachaki
Eythymia
E
Voulgarelis
Michalis
M
D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000209:etiology; D000974:Antibodies, Antinuclear / Q000276:immunology; D000918:Antibody Specificity; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology; D001853:Bone Marrow / Q000473:pathology; D002478:Cells, Cultured; D003114:Colony-Forming Units Assay; D003937:Diagnosis, Differential; D015672:Erythroid Precursor Cells / Q000276:immunology; D004920:Erythropoiesis / Q000276:immunology; D005260:Female; D006327:Heart Block / Q000151:congenital; D006801:Humans; D007231:Infant, Newborn; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D008232:Lymphoproliferative Disorders / Q000175:diagnosis; D012010:Red-Cell Aplasia, Pure / Q000209:etiology* / Q000276:immunology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000276:immunology
D000974:Antibodies, Antinuclear; D001323:Autoantibodies