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• W2090111624 abstract "Pure red cell aplasia (PRCA) is a rare haematological disorder characterized by selective marrow aplasia of the erythroid series. Although many cases of PRCA respond to immunosuppressive therapies, refractory cases are often observed. Fludarabine, a fluorinated purine analogue, has shown a significant activity against chronic lymphocytic leukaemia (CLL), as well as a strong immunosuppressive effect. We report the first case of PRCA that is not associated with CLL and has responded to fludarabine treatment. A 55-year-old woman was diagnosed with PRCA in 1985. She received immunosuppressive therapy with prednisolone, azathioprine and cyclophosphamide for 18 months and achieved transfusion independence. In 1995, the patient's disease deteriorated and she required regular red blood cell (RBC) transfusions, despite treatment with cyclosporin and prednisolone. When she presented to our hospital in April 1997, there was no peripheral lymphadenopathy or hepatosplenomegaly. Haemoglobin was 6·7 g/dl with reticulocytes of 0·09%. Leucocytes were 2·5 × 109/l (segmented neutrophils 40%, lymphocytes 53% and monocytes 6%), platelets were 187 × 109/l, serum ferritin was 5911 μg/l and the patient was negative for serum anti-nuclear antibody and rheumatoid factor. The bone marrow examination showed 25% cellularity with a myeloid:erythroid ratio of 21:1. A computerized tomogram of the chest showed no thymus enlargement or mediastinal lymphadenopathy. Her RBC transfusion requirement did not decrease despite trials with oxymetholone (50 mg/d) for 8 months, as well as a weekly dose of 4000 i.u. of erythropoietin for 3 weeks. Subsequently, anti-thymocyte globulin (ATG) 10 mg/kg was administered intravenously for 5 d with transient reticulocytosis lasting approximately 1 month (Fig 1). Further treatment with cyclosporin and prednisolone was not effective. Thereafter, the patient received three further courses of ATG therapy with only brief reticulocyte responses. The clinical course of the patient from April 1997 to August 2000. Haematological data are correlated with therapeutic trials as well as blood transfusion. Note reticulocyte and haemoglobin response after the treatment with fludarabine. Each small arrow (top) represents transfusion of 2 units of RBCs. CSA, cyclosporin; PD, prednisolone; ATG, anti-thymocyte globulin. Owing to continued dependence on RBC transfusion and a worsening of the symptoms of iron overload, such as malaise, darkening of the skin and hyperglycaemia, we decided to try a novel immunosuppressive agent. In April 2000, fludarabine (Fludara, Berlex Laboratories, Richmond, CA, USA) 30 mg/m2/d was administered intravenously for 4 d. No untoward reaction was observed. Two weeks later, the reticulocyte count rose to 6·16% from 0·07%. The haemoglobin level rose to 11·6 g/dl 7 weeks later (Fig 1). Bone marrow examination 10 weeks after the therapy showed an increase in the number of the erythroid series with myeloid:erythroid ratio of 5·5:1. The patient was independent of the RBC transfusion for over 4 months after fludarabine treatment. Her haemoglobin level remained over 11·0 g/dl. PRCA was reported to develop in as many as 6% of patients with CLL (Chikkappa et al, 1986). There are several reports showing resolution of CLL-associated PRCA after fludarabine treatment (Jaccard et al, 1993; Serra et al, 1999; Shimoni et al, 1999). Alternatively, Leporrier et al (1993) reported PRCA that developed during treatment of CLL with fludarabine. In contrast to the above-mentioned cases, there was no evidence that our patient had lymphoid malignancies. Any underlying indolent clonal lymphoid disorder would have manifested itself during the long duration of illness in our patient (15 years). In addition, there was no evidence of other immunological disorders such as connective tissue diseases or thymoma. Initial response to cytotoxic agents (8 years without RBC transfusion) and the brief response to ATG suggested that the underlying mechanism of PRCA in our patient was immune-mediated. After a course of treatment with fludarabine, our patient became independent of RBC transfusion and maintained her haemoglobin level above 11·0 g/dl for over 4 months. The immunosuppressive property of fludarabine is being explored for various clinical applications including graft-versus-host disease prophylaxis (Or et al, 2000) and non-myeloablative preparative regimens in allogeneic stem cell transplantation (Giralt et al, 1997). Further studies are warranted to investigate the role of fludarabine in the management of refractory idiopathic PRCA." @default.
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• W2090111624 date "2001-02-01" @default.
• W2090111624 modified "2023-09-26" @default.
• W2090111624 title "A case of refractory idiopathic pure red cell aplasia responsive to fludarabine treatment" @default.
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• W2090111624 doi "https://doi.org/10.1046/j.1365-2141.2001.02540.x" @default.
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