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Letters to the Editor
A
Case accrual 8 April - 6 May 2020
Date Age and sex of patients
B
Age (years)
Figure 1. Number of confirmed and suspected COVID-19 cases reported over a 4-week period from April 8 to May 6, 2020 (A) subdivided according to age and sex (B).
Patient Reporting System. In this large cohort the COVID- 19 related mortality rate in patients aged 18-49 years and 50-79 years was 194 of 8,764,368 (22.1 per million people) and 4,258 of 7,382,344 (576 per million people) respective- ly. Based on the number of patients registered on the NHR in the same age groups, equivalent figures for SCD are 4 of 5,242 (763 per million people) and 5of 1,682 (2,972 per million people) respectively. Cases without a laboratory confirmed COVID-19 diagnosis were excluded from the analysis. Comparison of the proportions of deaths in SCD and the general population indicates that SCD patients in the age groups 18-49 years and 50-79 years have an increased risk of COVID-19 related death, odds ratio (OR) 34.5 (range: 12.8-92.8, P<0.0001) and OR 4.1 (range: 1.5- 11.0, P=0.0047) respectively.
Patient characteristics and outcomes for thalassemia (n=26) are shown in Figure 2A and C. During the COVID- 19 episode 8 (31%) thalassemia patients received transfu- sion. Two deaths were reported, in patients with hemo- globin H disease and TDT aged 92 and 53 years. Both had concurrent morbidities in the form of cancer and splenec- tomy, iron overload and diabetes respectively. Only three patients with rare inherited anemia (unstable hemoglobin Hb Köln: 2, hereditary elliptocytosis: 1) were reported (Figure 2A). All received transfusion for acute hemolysis
during the COVID-19 episode and subsequently recov- ered. No cases of COVID-19 were reported in DBA.
As part of the survey HCC were encouraged to notify adverse events impacted by, though not necessarily direct- ly attributable to COVID-19. Two deaths were reported in SCD, in which delayed presentation may have contributed to the outcome. In one case there was confirmed gram- negative sepsis and in the other SARS-CoV-2 RNA was detected post-mortem.
Despite guidance to reduce the risk of SARS-CoV-2 infection in extremely vulnerable individuals by strict self- isolation (shielding) at home and government support with food and medicines, a significant number of hemo- globinopathy patients in the UK have developed COVID- 19. It is likely that the reasons for this are complex. Identification of extremely vulnerable patients initially relied on national digital health data systems. In a survey conducted by one of the participating centers around 20% of patients reported they had not received the initial shielding guidance issued by the NHS. Socioeconomic fac- tors such as low income and a need to preserve employ- ment, poor housing, and lack of practical support limit the ability of some households to sustain effective shielding measures. Furthermore, nosocomial SARS-CoV-2 infection is a risk for patients who have to maintain contact with
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haematologica | 2020; 105(11)
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