Pured red cell aplasia as a presentig form of systemic lupus erythmatosus
Abstract
Pure red cell aplasia (PRCA) is a syndrome defined by normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Occasionally it is triggered in the course of collagen or autoimmune diseases. We present the first case reported in the literature of a man with PRCA as the onset form of systemic lupus erythematosus (SLE). A 65-year-old man, who presented normocytic normochromic anemia, ANA 1/5120 and anti-Sm 2.61. He reported oral ulcers, polyarthralgia, swelling of both ankles and photosensitivity. Bone marrow examination showed red cell line hypoplasia due to maturation arrest at the level of the basophilic erythroblast, almost absence of mature cells, and a very high content of large proerythroblasts. With the diagnosis of PRCA as the first manifestation of SLE, he was successfully treated with Prednisone. We can conclude that screening for systemic diseases in patients with PRCA is essential to ensure correct management and a better prognosis.References
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