REAL-TIME NATIONAL SURVEY OF COVID-19 IN HEMOGLOBINOPATHY AND RARE INHERITED ANEMIA PATIENTS
Author(s): ,
Paul Telfer
Affiliations:
Haematology,Queen Mary University of London,London,United Kingdom
,
Josu De La Fuente
Affiliations:
Paediatric Haematology,Imperial College Healthcare NHS Trust,London,United Kingdom
,
Mamta Sohal
Affiliations:
Haematology,Imperial College Healthcare NHS Trust,London,United Kingdom
,
Ralph Brown
Affiliations:
Haematology,Imperial College Healthcare NHS Trust,London,United Kingdom;NIHR Imperial Biomedical Research Centre,London,United Kingdom
,
Perla Eleftheriou
Affiliations:
Haematology,University College London Hospitals NHS Foundation Trust,London,United Kingdom
,
Noemi Roy
Affiliations:
John Radcliffe Hospital,Oxford,United Kingdom;NIHR Biomedical Research Centre,Oxford,United Kingdom
,
Fred Piel
Affiliations:
School of Public Health, Faculty of Medicine,Imperial College,London,United Kingdom
,
Subarna Chakravorty
Affiliations:
King's College Hospital NHS Foundation Trust,London,United Kingdom
,
Kate Gardner
Affiliations:
Haematology,Guys and St Thomas's NHS Foundation Trust,London,United Kingdom
,
Mark Velangi
Affiliations:
Paediatric Haematology,Birmingham Children's Hospital,Birmingham,United Kingdom
,
Emma Drasar
Affiliations:
Haematology,Whittington Hospital,London,United Kingdom
,
Farrukh Shah
Affiliations:
Haematology,Whittington Hospital,London,United Kingdom
,
John Porter
Affiliations:
Haematology,University College London Hospitals NHS Foundation Trust,London,United Kingdom
,
Sara Trompeter
Affiliations:
Haematology,University College London Hospitals NHS Foundation Trust,London,United Kingdom
,
Wale Atoyebi
Affiliations:
Haematology,John Radcliffe Hospital,Oxford,United Kingdom
,
Richard Szydlo
Affiliations:
NIHR Imperial Biomedical Research Centre,Imperial College,London,United Kingdom
,
Kofie Anie
Affiliations:
Haematology,London North West University Healthcare NHS Trust,London,United Kingdom
,
Kate Ryan
Affiliations:
Haematology,Manchester University NHS Foundation Trust,Manchester,United Kingdom
,
Joseph Sharif
Affiliations:
Haematology,Manchester University NHS Foundation Trust,Manchester,United Kingdom
,
Josh Wright
Affiliations:
Haematology,Sheffield Teaching Hospital, Sheffield, UK,Sheffield,United Kingdom
,
Emma Astwood
Affiliations:
Paediatric Haematology,Sheffield Children’s Hospital,Sheffield,United Kingdom
,
Sarah Nicolle
Affiliations:
Haematology,University Hospitals Coventry and Warwickshire NHS Trust,Coventry,United Kingdom
,
Amy Webster
Affiliations:
Haematology,University Hospitals of Leicester,Leicester,United Kingdom
,
Sanne Lugthart
Affiliations:
Haematology,University Hospitals Bristol,Bristol,United Kingdom
,
Banu Kaya
Affiliations:
Haematology,Bart's Health NHS Trust,London,United Kingdom
,
Moji Awogbade
Affiliations:
Haematology,King's College Hospitals NHS Foundation Trust,London,United Kingdom
,
David Rees
Affiliations:
Paediatric Haematology,King's College Hospital NHS Foundation Trust,London,United Kingdom
,
David Roberts
Affiliations:
NHS Blood and Transplant,Oxford,United Kingdom
,
Robert Hollingsworth
Affiliations:
Medical Data Solutions and Services,Manchester,United Kingdom
,
Baba Inusa
Affiliations:
Paediatric Haematology,Guys and St Thomas's NHS Foundation Trust,London,United Kingdom
,
Jo Howard
Affiliations:
Haematology,Guys and St Thomas's NHS Foundation Trust,London,United Kingdom
Mark Layton
Affiliations:
Haematology,Imperial College Healthcare NHS Trust,London,United Kingdom;NIHR Imperial Biomedical Research Centre,London,United Kingdom
EHA Library. Telfer P. 06/14/20; 303394; LB2606
Paul Telfer
Paul Telfer
Contributions
Abstract
This abstract is embargoed until Saturday, June 13, 08:30 CEST.

Abstract: LB2606

Type: Oral Presentation

Presentation during EHA25: All oral abstract presentations will be made available on the on-demand Virtual Congress platform as of Friday, June 12 at 08:30 CEST and will be accessible until October 15, 2020.

Session title: Late-breaking Oral Session

Background
In March 2020 the UK Government advocated ‘shielding’ measures to protect extremely vulnerable patients deemed at high risk of COVID19.  On the advice of the National Haemoglobinopathy Panel, this guidance was extended to all sickle cell disease (SCD) sub-types, as well as to some patients with thalassaemia and rare inherited anemias. 

Aims
We undertook a national survey of confirmed and suspected COVID19 cases in haemoglobinopathies and rare anaemias to evaluate the impact of shielding and to guide clinical management and public health policy. 

Methods
From 8 April, data were submitted weekly by the 10 regional Haemoglobinopathy Coordinating Centres (HCCs) in England, following WHO case definitions for COVID19. Anonymised data were collected using a standardised report template, and data updated, analysed and reported back to the HCC's.  

Results
By 6 May 2020, a total of 195 cases (male: 86; female: 109) were reported. The median age was 33 years (range: 6 weeks to 92 years). There were 175 adults and 20 children (≤18 years). SCD accounted for 84% of cases (HbSS 63.6%; hemoglobin SC disease 15.4%; other phenotypes 5.2%) thalassemia disorders 12.7% (TDT 10.2%; NTDT 2.5%) and DBA and other rare anemias 3.1%. PCR for SARS-CoV-2 RNA was positive in 98 of 154 (64%) cases tested.  One hundred and forty three (74%) patients were admitted to  hospital and fifteen (10.5%), all adults, to critical care. Of these, 7 (4.9%) required non-invasive ventilation, 4 (2.8%) mechanical ventilation and 4 (2.8%) both. Outcome was analyzed for cases with a completed COVID19 course (N=168). Of these 155 (92.3%) have recovered and 13 (7.7%) died. Twenty-one (10.7% of cases reported) were receiving inpatient treatment as of 6 May 2020. Seven of 8 SCD patients who received mechanical ventilation have died with one patient receiving ongoing hospital treatment. 

No COVID19 hospital deaths occurred in children. Among hospitalized adults mortality was 9.8%. SCD accounted for 85% (11/13) of deaths.  The mortality rate in males was 6.9% (5/72) and in females 8.3% (8/96). Comorbid conditions recognized to increase the risk of death in COVID19 were identified in 7 (SCD 5; TDT 1; NTDT 1) out of 13 patients who died. The age-adjusted mortality for SCD patients with a completed course was 6% for patients below 50 years of age and 20.8% for those aged 50-69 years. 

Conclusion
Despite guidance to reduce the risk of COVID19 in extremely vulnerable individuals by strict self-isolation at home, a significant number of hemoglobinopathy patients have been infected with SARS-CoV-2. Socioeconomic factors and nosocomial transmission may have contributed to viral transmission. The low incidence of cases in children is commensurate with the generally mild course of COVID19 in this age group suggesting that shielding may not be necessary for all children with SCD and thalassemia. The increase of mortality with age is consistent with the known age demographic effect in COVID19. Patients with chronic organ damage may be at increased risk irrespective of age. Unexpectedly, mortality due to COVID19 did not show a clear correlation with clinical severity of the underlying condition or with gender.  Further analysis of accumulating data from the UK, pooled with other international registries may provide a clearer picture of the susceptibility to COVID19 and risk factors of severe outcomes in these patient groups.  In the meantime we advocate caution with respect to lifting of precautions implemented to protect these patients.

 

Session topic: 26. Sickle cell disease

Keyword(s): COVID-19, Hemoglobinopathy, Sickle cell disease, Thalassemia

This abstract is embargoed until Saturday, June 13, 08:30 CEST.

Abstract: LB2606

Type: Oral Presentation

Presentation during EHA25: All oral abstract presentations will be made available on the on-demand Virtual Congress platform as of Friday, June 12 at 08:30 CEST and will be accessible until October 15, 2020.

Session title: Late-breaking Oral Session

Background
In March 2020 the UK Government advocated ‘shielding’ measures to protect extremely vulnerable patients deemed at high risk of COVID19.  On the advice of the National Haemoglobinopathy Panel, this guidance was extended to all sickle cell disease (SCD) sub-types, as well as to some patients with thalassaemia and rare inherited anemias. 

Aims
We undertook a national survey of confirmed and suspected COVID19 cases in haemoglobinopathies and rare anaemias to evaluate the impact of shielding and to guide clinical management and public health policy. 

Methods
From 8 April, data were submitted weekly by the 10 regional Haemoglobinopathy Coordinating Centres (HCCs) in England, following WHO case definitions for COVID19. Anonymised data were collected using a standardised report template, and data updated, analysed and reported back to the HCC's.  

Results
By 6 May 2020, a total of 195 cases (male: 86; female: 109) were reported. The median age was 33 years (range: 6 weeks to 92 years). There were 175 adults and 20 children (≤18 years). SCD accounted for 84% of cases (HbSS 63.6%; hemoglobin SC disease 15.4%; other phenotypes 5.2%) thalassemia disorders 12.7% (TDT 10.2%; NTDT 2.5%) and DBA and other rare anemias 3.1%. PCR for SARS-CoV-2 RNA was positive in 98 of 154 (64%) cases tested.  One hundred and forty three (74%) patients were admitted to  hospital and fifteen (10.5%), all adults, to critical care. Of these, 7 (4.9%) required non-invasive ventilation, 4 (2.8%) mechanical ventilation and 4 (2.8%) both. Outcome was analyzed for cases with a completed COVID19 course (N=168). Of these 155 (92.3%) have recovered and 13 (7.7%) died. Twenty-one (10.7% of cases reported) were receiving inpatient treatment as of 6 May 2020. Seven of 8 SCD patients who received mechanical ventilation have died with one patient receiving ongoing hospital treatment. 

No COVID19 hospital deaths occurred in children. Among hospitalized adults mortality was 9.8%. SCD accounted for 85% (11/13) of deaths.  The mortality rate in males was 6.9% (5/72) and in females 8.3% (8/96). Comorbid conditions recognized to increase the risk of death in COVID19 were identified in 7 (SCD 5; TDT 1; NTDT 1) out of 13 patients who died. The age-adjusted mortality for SCD patients with a completed course was 6% for patients below 50 years of age and 20.8% for those aged 50-69 years. 

Conclusion
Despite guidance to reduce the risk of COVID19 in extremely vulnerable individuals by strict self-isolation at home, a significant number of hemoglobinopathy patients have been infected with SARS-CoV-2. Socioeconomic factors and nosocomial transmission may have contributed to viral transmission. The low incidence of cases in children is commensurate with the generally mild course of COVID19 in this age group suggesting that shielding may not be necessary for all children with SCD and thalassemia. The increase of mortality with age is consistent with the known age demographic effect in COVID19. Patients with chronic organ damage may be at increased risk irrespective of age. Unexpectedly, mortality due to COVID19 did not show a clear correlation with clinical severity of the underlying condition or with gender.  Further analysis of accumulating data from the UK, pooled with other international registries may provide a clearer picture of the susceptibility to COVID19 and risk factors of severe outcomes in these patient groups.  In the meantime we advocate caution with respect to lifting of precautions implemented to protect these patients.

 

Session topic: 26. Sickle cell disease

Keyword(s): COVID-19, Hemoglobinopathy, Sickle cell disease, Thalassemia

By clicking “Accept Terms & all Cookies” or by continuing to browse, you agree to the storing of third-party cookies on your device to enhance your user experience and agree to the user terms and conditions of this learning management system (LMS).

Cookie Settings
Accept Terms & all Cookies