A case of systemic lupus erythematosus accompanied with pure red cell aplasia

Abstract

Pure red cell aplasia (PRCA) is a rare cause of anemia associated with SLE. We herein report a case presenting with SLE and PRCA. A 33-year-old woman, who had been suffering from photosensitivity, proteinuria, and pancytopenia, was diagnosed to have SLE. She showed normochromatic normocytic anemia. The serum level of haptoglobin was <10 mg/dl, and Direct Coombs' test was negative. Her reticulocyte count was 0.8%. Her clinical and laboratory features, except for anemia, had recovered in response to 50 mg/day of prednisolone. The serum level of haptoglobin had normalized, but the reticulocyte count remained low. The bone marrow findings revealed erythroid hypoplasia, and she was diagnosed to have PRCA complicated with SLE. No viral DNA of human parvovirus B19 in her bone marrow was detected. The anemia gradually improved following the further use of 50 mg/day prednisolone. In order to disclose the mechanism of PRCA in this patient, we examined the effects of her peripheral T lymphocytes on erythrogenesis, using erythroid colony-forming cells (ECFC) in her peripheral blood. When we co-cultured peripheral T cells and ECFC, her T cells inhibited erythroid colony formation in a dose dependent manner. Several reports have shown the presence of inhibitory factors in SLE patients' serum such as antibodies against erythroid progenitors or erythropoietin, while other reports have shown abnormal T cells that inhibit the growth of erythroid progenitors. Our study suggests that these inhibitory T cells may therefore have played an important role in the pathogenesis of this patient.

Aplasia pura de células rojas como forma de presentación de lupus eritematoso sistémico

“…Hasta el momento la revisión de la literatura describe únicamente 13 casos en los que la APCR se diagnostica como debut o previo al inicio de los demás síntomas de LES (Tabla) [5][6][7][8][9][10][11][12][13][14][15][16] . Todos corresponden a mujeres, con una edad media de 30 años, siendo el caso actual el primero descripto de un varón, y además de edad avanzada (65 años).…”

Pure Red Cell Aplasia Associated with Systemic Lupus Erythematosus in Children Response to Rituximab– A Case Report and Literature Review

“…Pleurisy is less common in patients with concurrent PRCA [46], and instances of renal and central nervous system involvement, as well as serositis, appear to be infrequent [26,46]. Contrasting these observations, recent reports have identi ed renal complications characterized by signi cant proteinuria in this patient population [5,22,26,27,32]. A positive Coombs' test and autoimmune hemolytic anemia have also been documented in a subset of SLE with PRCA cases [40,46].…”