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. Am. J. Hematol. 72:259–262, 2003. © 2003 Wiley‐Liss, Inc.