P111: COVID-19 IN PATIENTS WITH THALASSEMIA AND SICKLE CELL DISEASE: A SINGLE CENTER EXPERIENCE
Authors: M Dimopoulou, V Komninaka, P Flevari, V Bartzi, K Repa, E Voskaridou
Journal: HemaSphere (2022)

Background: Thalassemic patients present complications such as iron overload, cardiac disease or diabetes that are expected to make them more vulnerable to COVID-19. Acute complications of SCD triggered by a viral infection include painful vaso-occlusive episodes, acute chest syndrome (ACS) and venous thromboembolic disease (VTE). Published data report variable rates of severe COVID-19 in thalassemia whereas for SCD more robust data exist suggesting a strong association with severe disease and mortality. Aims: The aim of the present study was to report the severity and mortality of COVID-19 in thalassemic and SCD patients of a Greek Center and investigate possible risk factors for severe disease. Patients and Methods: The patient population of our Center includes 200 thalassemic patients and 320 SCD (of which 73 and 53 transfusion dependent, respectively). Baseline clinical and laboratory data and manifestations, clinical course, treatment and outcome of COVID-19 were collected from medical files for all the patients that had a PCR-documented COVID-19 infection since the pandemic initiation. Association of characteristics with severe disease or hospitalization was investigated with Fisher’s exact test. Results: Since August 2020 until November 2021 34 patients (21 thal and 13 SCD) were infected by SARS-CoV2 corresponding to 10.5% and 4% of our patient population, respectively. Their median age was 47 yrs and 14 of them were male. Baseline patients characteristics as well as COVID-19 manifestations and outcome are presented on Table 1. None of the patient had cardiac iron overload, 2 had medium and 1 severe hepatic iron overload. Five patients had commorbidities (pulmonary hypertension 2, heart failure 1, atrial fibrillation 3, diabetes mellitus 2, arterial hypertension 1, malignant disease 1). Five patients had been vaccinated for COVID-19 before they presented with disease. The majority of patients (32/33) devepoped symptomatic disease of mild (26) or moderate (6) severity according to established criteria. Only one patient presented severe disease. Only 6/33 patients required hospitalization (4 thalassemic and 2 with SCD). In 4/6 hospitalized patients oxygen therapy with a nasal cannula was required and only one patient required a Venturi mask. None of the patients were intubated. One of the SCD patients developed an acute painful episode but there was no ACS post COVID-19. No increase in transfusion requirements was observed. There were no deaths in our patient population. All but one patients completely recovered whereas one SCD patient who was hospitalized is suffering from long-COVID. History of heart failure was associated with severe disease (p=0.03) and history of AF was associated with need for hospitalization (p=0.02). History of splenectomy, pulmonary hypertension or ACS and the degree of iron overload were not associated with disease severity or hospitalization requirement. Conclusion: COVID-19 had mild to moderate severity in the majority of our patients and only a minority of the patients required hospitalization. No ICU admissions or deaths were observed, comparing favourably to published data. Factors predisposing for severe COVID-19 in the general population, especially cardiac disease, seem to play a role also in patients with hemoglobinopathy. - BASELINE PATIENTS CHARACTERISTICS DIAGNOSIS TRANSFUSION DEPENDENT β-THALASSEMIA (TDT) 11 NON-TRANSFUSION DEPENDENT β-THALASSEMIA (NTDT) 7 HEMOGLOBINOPATHY H 2 SCD (ON CHRONIC TRANFUSIONS) 13 (4) TOTAL 33 AGE (YRS)* 47 (26–69) SEX (M/F) 14/17 SPLENECTOMY/FUNCTIONAL ASPLENIA 11/4 COMMORBIDITIES 5 SERUM FERRITIN* (ng/μl) 738 (49–2900) HYDROXYUREA TREATMENT 6 CHELATION THERAPY 14 MONOTHERAPY 10 COMBINATION 4 ANTICOAGULANTS 3 ANTI-PLATELETS 8 HISTORY OF ACS/MULTIPLE RELAPSES 3/1 HISTORY OF VTE 3 LIC (mg/gr)* 4.7 (1.1–21.4) ANTI-COVID VACCINATION 5 SEVERITY OF COVID INFECTION ASYMPTOMATIC 1 MILD (NO DYSPNEA) 27 MODERATE (DYSPNEA, SpO2 ≥94%) 5 SEVERE DYSPNEA, SpO2 <94%) 1 FEVER 23 COUGH 20 PAINFUL CRISIS 1 PULMONARY INFILTRATES 3 VTE 0 HOSPITALIZATION 6 TREATMENT ANTIBIOTICS 13 STEROIDS 6 REMDESIVIR 4 LMWH 7 anti-TNF 1 OXYGEN THERAPY 5 DEATHS 0 *:MEDIAN (RANGE) References 1. Mucalo L et al, Blood Adv. 2021; 5(13):2717–2724. 2. Clift AK, et al, Ann Intern Med. 2021; 174(10):1483–1487. 3. Vilela TS, et al, Hematol Transfus Cell Ther. 2021;43(1):87–100.