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Iron-deficiency anaemia is a widespread and largely preventable problem in the paediatric population, with numerous potential sequelae. We describe the case of a 2-year-old girl presenting with non-specific symptoms, who was found to be iron-deficient and anaemic, in the context of excessive cow's milk consumption. We explore the patient's diagnostic journey, including a neurological deterioration and the link between her iron deficiency and the final diagnosis. [ABSTRACT FROM AUTHOR]

Summary: Objectives: To determine the organisational resources in place; what blood was being transfused, why, how, where, when and by whom; whether laboratory support and policies met standards for patients with sickle cell disease (SCD). Background: SCD affects 14 000 people in the United Kingdom (UK). Standards and guidelines do not cover all aspects of transfusion in SCD and there are no data on their use; people may become very sick without warning presenting to non‐specialist hospitals; blood services are increasingly supplying units for transfusion in SCD with little data on their use. Methods: A retrospective audit of transfusion services/practice for people with SCD who had received a transfusion in January‐July 2014 in participating hospitals in the UK and Republic of Ireland (ROI). Results: Eighty‐four hospitals submitted 1290 cases, 75% of cases came from 18 hospitals submitting 25 or more cases. Transfusions (91.2% [1164/1276]) were administered to patients with HbSS, 60% (732/1227) of patients needed Rh CE negative blood. Transfusion episodes (4528) were recorded, of which 84% were elective. Stroke prevention accounted for 42% of all transfusions; adults received 56% of transfusions of which 50% were automated red cell exchange (RCE), children received 44% of transfusions of which 87% were simple transfusions. Conclusions: There was a paucity of appropriate clinical management protocols, adequately trained staff and network arrangements. The high numbers of children being transfused, disparity in transfusion modality between children and adults and the high frequency of the CE negative Rh phenotype were noted. [ABSTRACT FROM AUTHOR]

Summary: With the developing COVID‐19 pandemic, patients with inherited anaemias require specific advice regarding isolation and changes to usual treatment schedules. The National Haemoglobinopathy Panel (NHP) has issued guidance on the care of patients with sickle cell disease, thalassaemia, Diamond Blackfan anaemia (DBA), congenital dyserythropoietic anaemia (CDA), sideroblastic anaemia, pyruvate kinase deficiency and other red cell enzyme and membrane disorders. Cascading of accurate information for clinicians and patients is paramount to preventing adverse outcomes, such as patients who are at increased risk of fulminant bacterial infection due to their condition or its treatment erroneously self‐isolating if their fever is mistakenly attributed to a viral cause, delaying potentially life‐saving antibiotic therapy. Outpatient visits should be minimised for most patients, however some, such as first transcranial dopplers for children with sickle cell anaemia should not be delayed as known risk of stroke will outweigh the unknown risk from COVID‐19 infection. Blood transfusion programmes should be continued, but specific changes to usual clinical pathways can be instituted to reduce risk of patient exposure to COVID‐19, as well as contingency planning for possible reductions in blood available for transfusions. Bone marrow transplants for these disorders should be postponed until further notice. With the current lack of evidence on the risk and complications of COVID‐19 infection in these patients, national data collection is ongoing to record outcomes and eventually to identify predictors of disease severity, particularly important if further waves of infection travel through the population. [ABSTRACT FROM AUTHOR]

Summary: The International Collaboration for Transfusion Medicine Guidelines (ICTMG) has published guidance on transfusion for haemoglobinopathies. To give a UK perspective on this guidance, each of the recommendations in the ICTMG guideline were reviewed and the applicability for transfusion practice in the UK considered with reference to relevant published British Society for Haematology (BSH) guidelines and national standards. There was much consensus; however, there was disparity surrounding the recommendations for routinely extended matching in those with alloimmunisation. [ABSTRACT FROM AUTHOR]

Purpose To explore the use and reproducibility of magnetic resonance-derived myocardial T1 mapping in patients with iron overload. Materials and Methods The research received ethics committee approval and all patients provided written informed consent. This was a prospective study of 88 patients and 67 healthy volunteers. Thirty-five patients underwent repeat scanning for reproducibility. T1 mapping used the shortened modified Look-Locker inversion recovery sequence (ShMOLLI) with a second, confirmatory MOLLI sequence in the reproducibility group. T2* was performed using a commercially available sequence. The analysis of the T2* interstudy reproducibility data was performed by two different research groups using two different methods. Results Myocardial T1 was lower in patients than healthy volunteers (836 ± 138 msec vs. 968 ± 32 msec, P < 0.0001). Myocardial T1 correlated with T2* (R = 0.79, P < 0.0001). No patient with low T2* had normal T1, but 32% ( n = 28) of cases characterized by a normal T2* had low myocardial T1. Interstudy reproducibility of either T1 sequence was significantly better than T2*, with the results suggesting that the use of T1 in clinical trials could decrease potential sample sizes by 7-fold. Conclusion Myocardial T1 mapping is an alternative method for cardiac iron quantification. T1 mapping shows the potential for improved detection of mild iron loading. The superior reproducibility of T1 has potential implications for clinical trial design and therapeutic monitoring. J. Magn. Reson. Imaging 2015;41:1505-1511. © 2014 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Summary Red blood cell (RBC) antigen matching beyond ABO and RhD is commonly recommended for patients with sickle cell disease (SCD) and thalassaemia. We present an updated systematic literature review to inform evidence‐based guidelines on RBC matching. The Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) tool was used to develop recommendations. Six new observational studies (4 prospective, 2 retrospective) were identified. The six studies reported on 583 patients in total, including cross‐over designs, with sample sizes from 10 to 343. Studies were heterogeneous, utilising varying degrees of RBC matching and different definitions for ‘extended’ matching. All reported on alloimmunisation. One study reported on molecular matching. The reported prevalence of alloimmunisation using limited matching was 0%–50% and with extended matching was 0%–24%. Eighty‐two patients were alloimmunised before study entry. The risk of bias across studies was moderate to critical. The guideline panel recommends that ABO, RhDCcEe, and K‐compatible RBCs are selected for individuals with SCD and thalassaemia, even in the absence of alloantibodies, and that RBCs which are antigen‐negative to already existing clinically significant antibodies are chosen. There is a need for comparative research to define the benefit, impact, cost‐effectiveness, and feasibility of extended RBC matching strategies to prevent alloimmunisation. [ABSTRACT FROM AUTHOR]