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G. Ramsey 《Vox sanguinis》2017,112(7):648-659
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Disturbances in the physiological regulation of erythropoietin (EPO) in patients with sickle cell disease (SCD) may contribute to worsening anaemia and increased transfusion requirements, but the use of recombinant EPO in this group of patients is controversial. The objective of this study was to evaluate the use of this drug in adult patients with SCD and its effects on haemoglobin levels and transfusion requirements. We conducted a retrospective analysis at the University of Campinas, with nineteen adults with a diagnosis of SCD (HbSS and HbS/β+ thalassaemia), who had received at least 1 year of EPO therapy between 2007 and 2014. Haemoglobin concentrations and trends of variation in transfused RBC volumes were compared before and after EPO administration. We observed that seven patients had a good response to treatment (Hb increment higher than 1·5 g/dl) and nine had a partial response (0·5–1·5 g/dl increment) and there was a significant decrease in the need for transfusion amongst those who usually required regular transfusions. There were no increases in the rates of vaso‐occlusive crisis or venous thromboembolism in comparison to the year before the onset of the therapy. Erythropoietin therapy led to a marked increase in haemoglobin concentration with a concomitant decrease in the demand for transfusion. Considering all complications related to allogeneic transfusion, we believe that EPO therapy represents an important therapeutic tool in sickle cell anaemia.  相似文献   

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Hereditary haemochromatosis, one of the most common genetic disorders in the United States, can produce systemic iron deposition leading to end-organ failure and death if untreated. The diagnosis of this condition can be challenging as elevated serum ferritin may be seen in a variety of conditions, including acute and chronic liver disease, a range of systemic inflammatory states, and both primary and secondary iron overload syndromes. Appropriate and timely diagnosis of haemochromatosis is paramount as simple interventions, such as phlebotomy, can prevent or reverse organ damage from iron overload. The recognition of other aetiologies of elevated ferritin is also vital to ensure that appropriate intervention is provided and phlebotomy only utilized in patients who require it. In this review, we summarize the existing data on the work up and management of hereditary haemochromatosis and present a practical algorithm for the diagnosis and management of this disease.  相似文献   

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Lookback was initiated upon notification of an acute HBV infection in a repeat Irish donor, 108 days post‐donation. The donation screened non‐reactive by individual‐donation nucleic acid testing (ID‐NAT) using the Procleix Ultrio Elite multiplex assay and again when the archived sample was retested, but the discriminatory assay for HBV was reactive. The immunocompromised recipient of the implicated red cell component was tested 110 days post‐transfusion, revealing a HBV DNA viral load of 470 IU/ml. Genotype C2 sequences identical across two regions of the HBV genome were found in samples from the donor and recipient.  相似文献   

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It would be desirable to be able to distinguish fever as a result of febrile non‐haemolytic transfusion reactions (FNHTR) from other febrile conditions. To further characterize the inflammatory feature of FNHTR, we measured a large panel of inflammatory markers in pre‐ and posttransfusion plasma samples from patients with and without FNHTR following the transfusion of leucoreduced red blood cells. As FNHTR patients only displayed a significant increase in IL‐6, we conclude that changes in plasma cytokine levels during FNHTR are unlikely to be used diagnostically. An incidental finding of a distinct cytokine pattern in pretransfusion samples from FNHTR patients warrants further investigations, as it might be used to characterize the nature of FNHTR and to predict the risk of these adverse events.  相似文献   

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Background and objectives Severe anaemia is an important cause of mortality in developing countries. However, few studies have explored the use of and possibilities for blood transfusion services. The aims of this study are to explore the use of blood transfusion services at a hospital in sub‐Saharan Africa and to assess the quality of the transfusion services according to WHO guidelines. Materials and methods Patient age, gender, haemoglobin (Hb) level, diagnosis, hospital department and replacement donations were recorded for all blood transfusions administered at a district hospital in Malawi in January 2010. The laboratory equipment and procedures were scored according to WHO guidelines. Results The mean Hb of transfused patients was 4·8 g/dl. Fifty‐seven per cent (59/104) of the transfusions were given to children diagnosed with malaria, and 17% (18/104) were given to pregnant women. During the study period, blood was in stock and available for transfusion within 1 h of requisition. The equipment and procedures at this hospital met the main criteria for an adequate WHO stage of development. Conclusion In contrast to the advanced transfusion medicine in developed nations, our findings highlight the persistent and urgent need for life‐saving blood transfusions in especially young children and pregnant women in Africa. The results indicate that blood transfusion services adapted to local conditions may be a realistic solution for providing safe blood products in developing countries. Serious challenges, such as HIV transmission and sustainable organization of low‐risk blood donations should be addressed to assure access to safe blood products.  相似文献   

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During storage of red blood cells (RBC), these cells develop storage lesions. The clinical relevance of these storage lesions is heavily discussed in literature. In this review, different aspects of the storage lesion are shown and how these potentially affect posttransfusion performance of the RBC. An overview of the conflicting literature on the clinical relevance of prolonged storage is given, summarizing the evidence on associations with mortality, length of stay, (postoperative) infections and organ failure. Subsequently, possible explanations are given for the conflicting results in the clinical studies and suggestions on how to proceed.  相似文献   

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