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1/25. Acquired pure red cell aplasia--a case report.

    A married female patient of 36 years with chronic anaemia, because of pure erythroid aplasia with a haemolytic component and hypothyroidism due to antithyroid auto-antibodies, was subsequently discovered as a case of systemic lupus erythematosus (SLE). She was treated with corticosteroid and immunosuppressive therapy and her anaemia was corrected. The response of erythroid aplasia to corticosteroid and other immunosuppressive agents suggests that immunological factors play a role in erythroid aplasia in SLE. The occurrence of red cell aplasia in association with a variety of immune phenomenon supports the concept that in SLE, erythroid aplasia may be of immune aetiology.
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keywords = anaemia
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2/25. Autoimmune hemolytic anaemia with raised lupus parameters--a case report.

    patients with autoimmune hemolytic anaemia with raised lupus parameters may not present clinically with overt systemic lupus erythematosus (SLE) though a transitional form of the disease may exist between these two entities.
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ranking = 2.5
keywords = anaemia
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3/25. Excellent effect of steroid plus azathioprine in a young woman with pernicious anaemia and systemic lupus erythematosus.

    We describe a 29-year-old woman who developed pernicious anaemia 2 years after the diagnosis of systemic lupus erythematosus. This is a rare association despite the relationship between the autoimmune aetiologies of these two conditions. Seven other cases have been described, but our report demonstrates a case with an excellent response to steroid and azathioprine.
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ranking = 2.5
keywords = anaemia
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4/25. Anti-CD20 monoclonal antibody (Rituximab) for life-threatening autoimmune haemolytic anaemia in a patient with systemic lupus erythematosus.

    Innovative approaches are needed for patients with systemic lupus erythematosus (SLE) who develop autoimmune haemolytic anaemia (AIHA) that does not respond to conventional treatment. Rituximab, a chimaeric anti-CD20 monoclonal antibody, has been demonstrated to be highly effective for in vivo B-cell depletion. We report an 18-year-old-girl with SLE and life-threatening AIHA that did not respond to steroids, intravenous immunoglobulin and cyclosporin A. Rituximab was given weekly at 375 mg/m2 for two doses. The drug was well tolerated and the patient had no adverse effects. Her haemolytic disorder markedly ameliorated, with a progressive increase of haemoglobin levels, starting a few days after therapy. The patient remains disease-free 7 months later.
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ranking = 2.5
keywords = anaemia
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5/25. Systemic lupus erythematosus in children: a case report and review.

    A 12-year-old Papua New Guinean female presented initially with nonspecific clinical symptoms, fever of unknown origin and anaemia. She subsequently developed multisystem disease involving the respiratory, gastrointestinal, central nervous, musculoskeletal and cutaneous systems. She was diagnosed to have systemic lupus erythromatosus (SLE) and started on treatment. Unfortunately the patient defaulted from follow-up after treatment, which covered seven months only, to present with acute respiratory distress from which she died within 24 hours. A relevant literature review with the clinical features of systemic lupus erythematosus in children is described.
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ranking = 0.5
keywords = anaemia
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6/25. Mycophenolate mofetil as a treatment for autoimmune haemolytic anaemia in patients with systemic lupus erythematosus and antiphospholipid syndrome.

    Unresponsive autoimmune haemolytic anaemia (AIHA) may require therapy with second-line drugs. There is no consensus that any one of these agents is more effective than another. Mycophenolate mofetil (MMF) is an immunosuppressive drug proven to be effective in reducing renal allograft rejection as well as being used in autoimmune diseases including systemic lupus erythematosus (SLE). We describe its use in two patients who were treated with MMF for AIHA in the context of underlying SLE and antiphospholipid syndrome (APS). The patients showed a good response to treatment with MMF, suggesting a possible role in the treatment of AIHA.
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ranking = 2.5
keywords = anaemia
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7/25. Sub-clinical systemic lupus erythematosus presenting with acute myocarditis.

    A 46 year old woman presented with fever and normochromic anaemia followed rapidly by severe myocardial failure, unresponsive to maximum inotropic support and broad spectrum antibiotics. There were no classical clinical stigmata of systemic lupus erythematosus (SLE) but a possible immunological cause was looked for, and on the basis of her immuno-serology a diagnosis of SLE-like disease was made. She responded rapidly to high dose steroids. The importance of considering the possibility of SLE or 'lupus overlap' in an acutely ill 'undiagnosed' patient is emphasized. The relevance of instigating appropriate immuno-serological tests in the course of such an illness is discussed.
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ranking = 0.5
keywords = anaemia
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8/25. Management of therapy-resistant systemic lupus erythematosus with rituximab: report of a case and review of the literature.

    Therapy of systemic lupus erythematosus (SLE) with major organ involvement consists of aggressive immunosuppression with glucocorticoids and cytotoxic agents. When remission is achieved, maintenance therapy is begun to reduce the risk of relapse while minimizing toxicity. Remission with standard therapy is, however, not always achieved. We discribe a women with SLE and microangiopathic haemolytic anaemia and thrombocytopenia, pneumonitis and nephritis refractory to high-dose steroids, pulse cyclophosphamide, plasmapheresis and intravenous immunoglobulins. The anti-CD20 monoclonal antibody rituximab was administered, resulting in major clinical and biochemical improvement. Therapy-resistant SLE generally has an ominous prognosis. A few anecdotal reports and small open studies describe beneficial effects of rituximab in these cases. Rituximab may be a promising new approach to improve the dismal outcome of therapy-resistant SLE.
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ranking = 0.5
keywords = anaemia
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9/25. Histiocytic necrotising lymphadenitis in systemic lupus erythematosus.

    Histiocytic necrotising lymphadenitis is the pathognomonic histological appearance of lymph nodes in Kikuchi's disease, a condition characterised by a brief systemic illness and lymphadenopathy. The case is described of a young man, originally diagnosed as having Kikuchi's disease by lymph node histology, who subsequently developed systemic lupus erythematosus with symmetrical polyarthritis, Coombs' positive haemolytic anaemia and haemorrhagic pneumonitis. The case emphasises that a range of diseases is associated with histiocytic necrotising lymphadenitis, belying the unitary impression given by the term Kikuchi's disease.
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ranking = 0.5
keywords = anaemia
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10/25. Significance of autoimmune haemolytic anaemia and immune thrombocytopenia (Evans' syndrome) in systemic lupus erythematosus.

    patients with systemic lupus erythematosus (SLE) have an extremely variable prognosis and mortality. The purpose of this report is to highlight the importance of looking for lupus parameters in patients with autoimmune haemolytic anaemia (AIHA) and/or immune thrombocytopenia (ITP) as this represents a subgroup of systemic lupus erythematosus (SLE) patients with a fairly good prognosis. We report a case of an 8-year-old Malay boy who was admitted to hospital with fever and gum bleeding. physical examination revealed a malar rash, oral ulcers, petechial haemorrhages and bruises over the limbs, generalised lymphadenopathy and hepatosplenomegaly. Laboratory investigations confirmed the diagnosis of SLE. The patient's serum showing the presence of antinuclear factor, antiphospholipid antibodies and a biological false-positive test for syphilis. Immunological and haematological parameters were in keeping with combined AIHA and ITP (Evans' syndrome). No organ involvement was present and the patient responded well to corticosteroid therapy. This case demonstrates the importance of making an early diagnosis of SLE with haematological complications, in order to ensure full benefit of therapy and emphasises the good prognosis expected in this subgroup of SLE patients.
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ranking = 2.5
keywords = anaemia
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