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Letters to the Editor
Consistent with our findings both studies observed a dis- proportionate number of COVID-19 rela-ted deaths in mild SCD genotypes. Differing conclusions were drawn with respect to overall morbidity and mortality of COVID- 19 in SCD. This highlights the importance of population- based risk estimates which neither study included. In this regard the preliminary results reported here suggest COVID-19 is associated with increased mortality in adults with SCD. Further studies are needed to refine the magni- tude of risk and determine the extent to which it reflects an independent effect or association with other clinical, demographic or socioeconomic risk factors. Current evi- dence suggests patients with sickle-related chronic organ damage may be at increased risk irrespective of age and underpins a ratio-nale for individual risk assessment in shielding guidance. This has important implications for prevention as European countries proceed with a phased easing of lockdown measures in the population.
In thalassemia and rare anemias, only tentative conclu- sions can be drawn given the small number of cases reported. In the former, outcomes were in keeping with an initial report from Italy, which indicates the course of COVID-19 in thalassemia is generally favorable.11 Both thalassemia cases associated with a fatal outcome in our survey had independent predictors of mortality.
HCC were instructed to notify both proven and clinical- ly suspected cases of COVID-19, in contrast to the re- gistry studies reported. In the early stages of the pandemic, laboratory capacity in the UK was limited and many patients with clinical features of COVID-19, particularly those with milder disease, were not tested. The limited sensitivity of RT-PCR testing for SARS-CoV-2 infection, with false negative rates up to 30%,12 was a further consid- eration in our decision to include clinically suspected cases. In order to avoid ascertainment bias the use of WHO criteria for suspected cases was recommended. Nevertheless, this may have led to an overestimation of cases which, with the availability of testing for antibodies to SARS-CoV-2, it will be possible to evaluate retrospec- tively. Although all HCC were invited to participate not all returned complete data due to workforce constraints or competing priorities during the pandemic. HCC have been encouraged to retrospectively enter data. This will enable a complete analysis to be reported in due course, allowing estimates of incidence and morbidity to be refined.
Although not its primary purpose, our survey raises con- cern that an unintended consequence of shielding could be the delayed presentation of life-threatening complications in hemoglobinopathy and rare anemia patients. Observational studies to measure excess deaths in vulner- able patient groups during the COVID-19 pandemic are needed. In order to mitigate this risk the NHP in collabo- ration with NHS England and patient support groups issued guidance that while shielding patients should con- tinue to access normal pathways for managing complica- tions of their condition and notify their center of care immediately if they develop suspected COVID-19 symp- toms to ensure presentations which overlap, inclu-ding bacterial infection and acute chest syndrome, are recog- nized and treated promptly.2 Our national survey, under- taken in a real-world setting, expands on case series from single centers13,14 and complements recently repor-ted reg- istry data from France and the USA in contributing to an understanding of the direct and collateral impact of COVID-19 in SCD and other inherited anemias.
Paul Telfer,1 Josu de la Fuente,2,3 Mamta Sohal,2 Ralph Brown,2 Perla Eleftheriou,4 Noémi Roy,5,6
Frédéric B. Piel,7 Subarna Chakravorty,8 Kate Gardner,9 Mark Velangi,10 Emma Drasar,4,11 Farrukh Shah,4,11
John B. Porter,4 Sara Trompeter,4,12 Wale Atoyebi,5
Richard Szydlo,13 Kofi A. Anie,14 Kate Ryan,15 Joseph Sharif,15 Josh Wright,16 Emma Astwood,17 C. Sarah Nicolle,18
Amy Webster,18 David J. Roberts,5,6,20 Sanne Lugthart,21 Banu Kaya,1 Moji Awogbade,8 David C. Rees,8
Rob Hollingsworth,22 Baba Inusa,9 Jo Howard9 and
D. Mark Layton2,3 for the Hemoglobinopathy Coordinating Centers and National Hemoglobinopathy Panel, England
1Barts Health NHS Trust, London; 2Imperial College Healthcare NHS Trust, London; 3NIHR Imperial Biomedical Research Centre, London; 4University College London Hospitals NHS Foundation Trust, London; 5John Radcliffe Hospital, Oxford; 6NIHR Biomedical Research Centre, Oxford; 7School of Public Health, Faculty of Medicine, Imperial College London, London; 8King's College Hospital NHS Foundation Trust, London; 9Guy’s and St Thomas' NHS Foundation Trust, London; 10Birmingham Children's Hospital, Birmingham; 11Whittington Health NHS Trust, London; 12NHS Blood and Transplant, London; 13Imperial College, London; 14London North West University Healthcare NHS Trust, London; 15Manchester University NHS Foundation Trust, Manchester; 16Sheffield Teaching Hospital, Sheffield; 17Sheffield Children’s Hospital, Sheffield; 18University Hospitals Coventry and Warwickshire NHS Trust, Coventry; 19University Hospitals of Leicester, Leicester; 20NHS Blood and Transplant, Oxford; 21University Hospitals Bristol, Bristol, UK and 22Medical Data Solutions and Services, Manchester, UK
Correspondence:
MARK LAYTON - m.layton@imperial.ac.uk
doi:10.3324/haematol.2020.259440
References
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haematologica | 2020; 105(11)