Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pure red cell aplasia is a selective aplasia of the marrow erythroid cells. Unlike aplastic anemia, the marrow has a normal cellularity and the patients generally have normal leukocyte and platelet blood counts. The congenital form of the disease occurs in the firlst 1 1/2 years of life and is often responsive to corticosteroids. The acquired form may be secondary to infections, drugs, chemicals, or hemolytic anemia (aplastic crisis). In these cases it is often acute and self-limited with cessation of the infection or drug ingestion. It may also be secondary to systemic lupus erythematosus, rheumatoid arthritis, acute severe renal failure, severe nutritional deficiency, or diverse neoplasms, and may remit with treatment of the primary condition. When a thymoma is present, it should be resected since a remission is produced in 29 per cent of these patients. The remaining patients have an acquired primary form of the disease that tends to be chronic and in some cases may have an immune pathogenesis. A cytotoxic immunoglobulin inhibitor of the marrow erythroid cells or erythropoietin has been described and these patients may respond to prednisone and/or to cytotoxic immunosuppressive drugs such as cyclophosphamide and 6-mercaptopurine. Pure red cell aplasia appears to be more common than the literature has revealed and has stimulated much investigation into an immune pathogenesis for marrow failure.
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PMID:Diagnosis and treatment of pure red cell aplasia. 78 16

We describe a patient with mixed connective tissue disease (MCTD), who developed pure red cell aplasia which responded favorably during treatment with corticosteroids. Pure red cell aplasia has been described in a few patients with rheumatoid arthritis and systemic lupus erythematosus, but, to our knowledge, this is the first report of an association between it and MCTD.
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PMID:Pure red cell aplasia in mixed connective tissue disease. 250 97

Pure red cell aplasia developed in a female patient with systemic lupus erythematosus (SLE). Erythroid colony growth was assessed in semisolid medium culture of bone marrow obtained from a normal donor and cultured in the presence of normal and patient sera. Colony forming units of erythropoiesis and burst forming units of erythropoiesis obtained from a normal donor were inhibited in the presence of patient sera. Our findings support the concept that circulating inhibitors might influence the proliferation of erythroid progenitor cells and erythroid aplasia may be an immunologically mediated syndrome.
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PMID:Pure red cell aplasia in a patient with systemic lupus erythematosus. 313 12

We describe a patient who developed pure red cell aplasia as part of a lupus-like syndrome while taking procainamide. Pure red cell aplasia resolved spontaneously after discontinuation of this drug. Although pure red cell aplasia has been reported in several patients with idiopathic systemic lupus erythematosus (SLE), this represents the first such report in a patient with drug induced SLE.
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PMID:Pure red cell aplasia in procainamide induced systemic lupus erythematosus. Report and review of the literature. 314 34

Pure red cell aplasia (PRCA) is an unusual early manifestation of systemic lupus erythematosus (SLE). Review of the 13 cases in literature revealed that the immune system played a significant role in disease pathogenesis. Immunosuppression has been thought to be the cause of the efficacy of corticosteroids in treatment. The case of a 28-year-old woman with SLE is described. She presented with PRCA, but has had good response to corticosteroid therapy.
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PMID:Systemic lupus erythematosus with pure red cell aplasia: a case report. 825 21

Pure red cell aplasia is characterized by severe anemia, with reticulocytopenia and absence of precursor cells in the bone marrow. Many modes of treatment have been described, including the use of immunosuppressive agents. Recently repeated courses of high-dose intravenous immunoglobulins have been used successfully in patients with idiopathic pure red cell aplasia. We here describe a 22-year-old woman who developed pure red cell aplasia in the course of systemic lupus erythematosus. After failure of corticosteroid therapy the patient was treated with one course of high-dose intravenous immunoglobulins with complete remission. No further therapy was required.
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PMID:Pure red cell aplasia associated with systemic lupus erythematosus: remission after a single course of intravenous immunoglobulin. 836 4

Pure red cell aplasia (PRCA) is a rare cause of anemia associated with systemic lupus erythematosus (SLE), and fewer than 20 cases have been reported. The development of PRCA may be mediated by an autoimmune mechanism which is supported by the presence of antibodies that impair various stages and mechanisms of erythropoiesis, by the association with immunological disorders or lymphoma, and by a favorable response to immunosuppressive drugs, antilymphocyte globulin, thymectomy, and splenectomy. However, these therapies have not been successful in all patients with PRCA. We report our experience with a 31-year-old female patient with SLE who developed PRCA that did not respond to immunosuppressive therapies. However, complete normalization of erythropoiesis was achieved after the removal of the autoantibodies by plasmapheresis, and the patient has now maintained a normal hemoglobin level for more than eight months. We suggest that plasmapheresis might be tried in the treatment of PRCA cases before other more aggressive therapies are commenced.
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PMID:Successful treatment of pure red cell aplasia with plasmapheresis in a patient with systemic lupus erythematosus. 1197 Dec 24

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.
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PMID:[A case of systemic lupus erythematosus accompanied with pure red cell aplasia]. 1681 63

Pure red cell aplasia (PRCA) is a rare hematologic disorder characterized by a severe progressive anemia and a marked decrease or absence of red cell precursors on bone marrow examination. PRCA can occur in the course of many other diseases of which thymoma is the most common. Rarely, PRCA has occurred in patients with connective tissue disorders, the most common being systemic lupus erythematosus (SLE), with 13 cases reported to date in the English literature. In this subset of patients, PRCA can be difficult to manage, and a high rate of mortality has been reported. Multiple therapeutic modalities have been reported, with no clear consensus on the optimal treatment. To date, splenectomy has not been reported in this subset of patients.We describe a patient with SLE who developed PRCA. Prednisone was the initial treatment, but unacceptably high doses were required to control the disease. Intravenous gamma globulin was given with prompt but temporary improvement. The patient subsequently underwent splenectomy with resolution of the PRCA. The patient continues to have a normal hemoglobin 3 years after splenectomy despite no interim therapy. This case represents the 14th case of PRCA occurring in patients with SLE and is the first case of PRCA in SLE treated with splenectomy. Splenectomy should be considered as a therapeutic modality in the management of corticosteroid-refractory PRCA occurring in patients with SLE.
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PMID:Pure red cell aplasia in systemic lupus erythematosus. 1907 27

A 47-year-old woman who had been treated for systemic lupus erythematosus (SLE) with myasthenia gravis (MG) was admitted to our hospital with acute onset of severe anemia after administration of isoniazid. Pure red cell aplasia (PRCA) was confirmed by elevated serum iron levels, reticulocytopenia and bone marrow aspiration showing a remarkable reduction of erythroblasts. Finally, cyclosporine A successfully improved PRCA. Although both SLE and MG have the potential complication of PRCA, we report here a case of isoniazid-triggered PRCA.
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PMID:Isoniazid-triggered pure red cell aplasia in systemic lupus erythematosus complicated with myasthenia gravis. 1970 20


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