Page 203 - 2020_11-Haematologica-web

CASE REPORTS
COVID-19 in patients with sickle cell disease – a
case series from a UK tertiary hospital
At the time of this manuscript going to press, Europe remains the epicenter of the COVID-19 pandemic and new cases and deaths in the UK continue to demonstrate an exponential rise.1 London has the highest number of reported UK cases.2 Clinical reports indicate that older adults with comorbidities such as diabetes and hyperten- sion are most at risk of severe COVID-19.3,4 Overwhelming inflammation and cytokine associated lung injury are potential pathological features. Secondary hemophagocytic lymphohistiocytosis-like syndrome with raised pro-inflammatory cytokines has been associ- ated with adverse outcomes.5
King’s College Hospital is a teaching hospital in South London, caring for approximately 500 adults and 500 children with sickle cell disease (SCD). South London currently has some of the highest numbers of confirmed cases of COVID-19 in England2 and a large local SCD cohort. It is thought that patients with SCD might demonstrate a more severe illness if infected with SARS- CoV-2 due to associated functional hyposplenia, high prevalence of concomitant chronic respiratory disease and increased levels of inflammation.6
In this report we describe the clinical features of the first 10 confirmed cases of COVID-19 in patients with SCD in the King’s College Hospital. At the time of this report, there were 22,141 confirmed cases of COVID-19 in the UK, of which the majority of cases were from the boroughs of Lambeth and Southward in South London.2 All patients underwent real-time quantitative PCR assay from RNA extracted from nasopharyngeal swabs using a locally validated procedure recommended by Public Health England.7 All patients had homozygous SCD (HbSS) and presented with symptoms such as cough, fever, coryza and associated acute sickle vaso-occlusive pain. None had any recent travel history (Table 1).
Apart from patient 9 who has severe pre-morbid di- sease with intensive care admission within the last 12 months due to SCD-related cerebrovascular disease, all patients had relatively mild clinical symptoms related to COVID-19 (Table 2).
In this series, seven patients were female, and the median age was 37 years (range: 25-54 years). No chil- dren were seen with SCD and COVID-19. All but two patients were on some disease modification treatment, either hydroxycarbamide (2 of 10) or transfusions (6 of 10). Patients on top-up transfusions were on a 4-weekly program with a post-transfusion hemoglobin target of 120-130 g/L. One patient was on an angiotensin convert- ing enzyme inhibitor (patient 6), and this was not discon- tinued during the period of illness. Two patients had a history of overt strokes and one patient had a history of recurrent transient ischemic attacks. All patients had on- going comorbidities, ranging from end stage renal failure to hyperhemolysis. All admitted patients received stan- dard thromboprophylaxis with low molecular weight heparin injection as per hospital venous thromboem- bolism prevention guidelines.
The mean number of days from onset of symptoms to PCR testing was 2.5 days. Of the seven patients in this cohort needing hospital admission, the mean number of days from the onset of symptoms to hospital admission was two days. Nine of 10 patients made a full recovery. Two patients presenting with cough and hypoxia received early top up transfusions. See Figure 1 for the chest radiograph of patient 2 who was hypoxic on admis- sion and received an additive transfusion. All in-patients received broad spectrum antibiotics to cover community acquired pneumonia. No COVID-19 specific treatment was given. The lymphocyte count fell significantly during infection compared to the baseline, from a median of 3.7 to 1.9 x109/L (P=0.037, Wilcoxon signed-rank test).
One patient died of respiratory complications follow- ing COVID-19. She had multiple comorbidities, inclu-
Table 1. Characteristics of COVID-19 positive patients with sickle cell disease.
Patient Genotype Sex number
1 HbSS M
2 HbSS F
3 HbSS F
4 HbSS F
5 HbSS M
6 HbSS F
7 HbSS M
8 HbSS F
9 HbSS F
10 HbSS F
Age Baseline Hb Baseline Smoker (years) concentration HbF
(g/L) percentage
36 - 5.2 N 38 87 3.2 N
34 106 0.8 N
46 91 1.5 N
37 104 0.4 N 52 115 18 N 25 114 0.8 N 35 97 19.6 N 54 105 11.9 N
44 108 0.9 N
BMI HC
26.9 N
40.8 N 20.9 N
25.7 N
Regular Comorbidity transfusions
Topup ACSinthelast12 months, chronic pain
24 N 28.3 Y 25.8 N
Topup ARECT
Topup
ARCET N ARCET
Recurrent leg ulcers Stroke, severe cerebral vasculopathy ESRF, HDx, Chronic pain, asthma Stroke Chronic shoulder pain Recurrent TIA
- Y 33.4 N
N Chronichippain N Hyperhemolysis, asthma, bilateral
hip AVN
ARCET ACSinthelast12
months, stroke,
iron overload
haematologica | 2020; 105(11)
31.5 N
ACS:acute chest syndrome,ARCET:automated red cell exchange transfusion,AVN:avascular necrosis,Hb:hemoglobin,HbF:fetal hemoglobin,ESRF:end stage renal failure, HDx: hemodialysis, HbSS: homozygous sickle cell disease, TIA: transient ischaemic attack, HC: hydroxycarbamide.
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