Table 3.
Case reports of patients with SCD and COVID-19. Authors listed in alphabetical order.
| Authors | Country | Sample size n (M/F) |
Age (years) | Hb genotype (n) | SCD therapy (n) |
Main findings | Hosp. rate n (%) |
Mortality rate n (%) |
|---|---|---|---|---|---|---|---|---|
| AbdulRahman et al. [102] | Bahrain | 6 (4/2) | 31 | HbSS (6) | HU (6) | Infection rate, clinical course, and viral clearance of SCD pts. were no different from pts. w/o SCD. | n/a | 0 (0) |
| Al-Hebshi et al. [75] | Saudi Arabia | 2 (1/1) | 13 | HbSS (2) | HU (2) BT (1) |
VOC and ACS complicated infection but were effectively treated according to national guideline and standard practice. | n/a | 0 (0) |
| Allison et al. [67] | USA | 1 (1/0) | 27 | HbSC (1) | BT (1) | Early red blood cell exchange treatment may have helped mitigate COVID-19 pneumonia and ACS. | n/a | 0 (0) |
| Al-Naami et al. [41] | Saudi Arabia | 3 (2/1) | 25 | HbSS (3) | HU (1) | Disease course was mild to moderate w/o complications or death. | 2 (67) | 0 (0) |
| Al-Naami et al. [103] | Saudi Arabia | 1 (1/0) | 29 | HbSS/Sβ0 | HU (1) | Pt presented asymptomatic with h/o sore throat and loose motions; Uneventful recovery. | n/a | 0 (0) |
| Al Sabahi et al. [76] | Oman | 5 (4/1) | 5 | HbSS/HbSβ+ (4/1) | HU (2) BT (4) |
Variable disease courses. All cases recovered. | 4 (80) | 0 (0) |
| Al Yazidi et al. [104] | Oman | 7 (n/a) | n/a | n/a | BT (2) | Most pts. had SCD-related symptoms, including ACS, VOC, and splenic sequestration. Three required supplemental oxygen. | n/a | 0 (0) |
| André et al. [77] | France | 1 (0/1) | 5 | n/a | n/a | ICU admission for rapid respiratory degradation after initial phase with mild symptoms. | n/a | 0 (0) |
| Anusim et al. [42] | USA | 11 (4/7) | 44 | HbSS (5), HbSC (4), HbSβ+ (1), HbSα (1) | BT (5) | Pt presentations varied from mild to severe; Older age/milder genotypes had worse outcomes; ACS and COVID-19 pneumonia present similarly and should be differentiated before treatment. | 10 (91) | 2 (18) |
| Appiah-Kubi et al. [78] | USA | 7 (2/5) | 14 | HbSS (6), HbSC (1) |
HU (4) BT (1) |
One ICU stay, no intubations. Favorable outcomes were due to early diagnosis, use of antivirals, anti-inflammatory agents, and anticoagulants. | 4 (57) | 0 (0) |
| Argüello-Marina et al. [105] | Spain | 7 (n/a) | n/a | n/a | n/a | Two ICU admissions, one patient required ventilation. Variable medications and treatments used. Overall, patients had short lengths of stay and no deaths. | 5 (71) | 0 (0) |
| Azerad et al. [106] | Belgium | 3 (1/2) | 30 | HbSS (3) | HU (3) BT (3) |
SCD pts. showed mild clinical presentation of COVID-19, which can be misleading; If BT is performed, always consider risk of DHTR. | n/a | 0 (0) |
| Beerkens et al. [54] | USA | 1 (1/0) | 21 | HbSβ0 (1) | HU (1) BT (1) |
COVID-19 may have caused or exacerbated ACS. | n/a | 0 (0) |
| Chakravorty et al. [43] |
UK | 10 (3/7) | 37 | HbSS (10/10) | HU (2) BT (6) |
All but one seemed to be experiencing a relatively mild course despite significant comorbidities. | 7 (70) | 1 (10) |
| Chen-Goodspeed et al. [44] |
USA | 5 (3/2) | 31 | HbSS (4), HbSC (1) | HU (3) BT (2) |
SCD pts. did not present typical COVID-19 symptoms but rather more typical SCD symptoms, including VOC; SCD pts. presenting with VOC should immediately be tested for COVID-19. | 3 (60) | 0 (0) |
| Dagalakis et al. [79] | USA | 1 (1/0) | 0.5 | HbSC | n/a | Pediatric pt. presented with mild symptoms and remained stable throughout hospitalization. | n/a | 0 (0) |
| De Luna et al. [107] | France | 1 (1/0) | 45 | HbSS | BT (1) | Severe pneumonia and severe ACS effectively treated with hydroxychloroquine and tocilizumab. | n/a | 0 (0) |
| Elia et al. [[56]] | Brazil | 3 (1/2) | 11 | HbSS (2), HbSC (1) | HU (2) | SCD pts. showed better clinical evolution than predicted; Pulmonary viral infections can trigger SCD symptoms and need for hospitalization; COVID-19 symptoms are very similar to ACS symptoms, which affects clinical decision. | n/a | 0 (0) |
| Ershler et al. [108] | USA | 1 (0/1) | 39 | HbSS | BT (1) | Acute crisis and COVID-19 pneumonia effectively treated with voxelotor in lieu of single BT. | n/a | 0 (0) |
| Espanol et al. [80] | USA | 1 (0/1) | 8 | HbSS | HU (1) BT (1) |
Patient was successfully managed after developing severe ACS secondary to COVID-19, complicated by cortical vein thrombosis and multisystem inflammatory syndrome. | n/a | 0 (0) |
| Fronza et al. [109] | Italy | 1 (0/1) | 44 | n/a | BT (1) | Patient recovered and was discharged after severe anemia and acute lung failure. | n/a | 0 (0) |
| Hardy et al. [110] | Ghana | 3 (0/3) | 28 | HbSS (1), HbSC (2) |
BT (2) | One had severe COVID-19 pneumonia. Two had severe SCD crisis. All had favorable outcomes. | n/a | 0 (0) |
| Heilbronner et al. [81] | France | 4 (1/3) | 15 | HbSS (4) | HU (2) BT (4) |
All pts. presented with ACS and admitted to PICU. Pts were effectively treated with erythrapheresis, NIV, and typical supportive treatment. | n/a | 0 (0) |
| Hussain et al. [55] | USA | 4 (2/2) | 33 | HbSS (2), HbSC (1), HbSβ+ (1) |
BT (1) | All four pts. initially presented to the ED for typical VOC; the clinical course of their COVID-19 infection was rather mild. | n/a | 0 (0) |
| Jacob et al. [82] | USA | 1 (1/0) | 2 | HbSS | HU (1) BT (1) |
Pediatric pt. with severe anemia, splenic sequestration crisis and COVID-19; Pt greatly improved following BT. Mild clinical course during hospitalization, with no apparent morbidity related to COVID-19. | n/a | 0 (0) |
| Justino et al. [111] | Brazil | 1 (0/1) | 35 | HbSS | BT (1) | Despite previous h/o pulmonary disease and current pregnancy, clinical course was very favorable. | n/a | 0 (0) |
| Kasinathan et al. [83] | USA | 1 (0/1) | 20 | HbSC | BT (1) | SCD diagnosis was unknown at time of presentation. Pulmonary embolism complicated condition. | n/a | 0 (0) |
| Martone et al. [84] | USA | 1 (0/1) | 19 | HbSβ0 | BT (1) | PT developed ACS with fever, decreased hemoglobin, but no hypoxemia, which was managed with BT and antibiotics. Four weeks later, pt. was re-hospitalized with post-COVID-19 encephalopathy, hyperammonemia, hyperinflammation, and multi organ failure. Patient recovered to neurologic baseline and normal liver function. | n/a | 0 (0) |
| McCloskey et al. [53] |
UK | 10 (8/2) | 36 | HbSS or HbSβ0 (9), HbSC (1) |
BT (5) | Overall, the outcome of COVID-19 infection was favorable for this cohort of SCD pts., unless there were significant comorbidities. | n/a | 1 (10) |
| Morrone et al. [73] | USA | 8 (4/4) | 16 | HbSS (7), HbS HPFH (1) |
HU (3) BT (3) |
SCD pts. not admitted and did not develop ACS were on HU and had lower absolute monocyte counts compared to those admitted and developed ACS. | 5 (63) | 0 (0) |
| Nur et al. [112] | Netherlands | 2 (1/1) | 22 | HbSS (2) | n/a | COVID-19 might trigger VOC w/o typical flu-like symptoms; ACS developed w/o typical COVID-19 pulmonary complications; SCD pts. presenting with VOC should be tested for COVID-19. | n/a | 0 (0) |
| Nguyen et al. [113] | USA | 1 (0/1) | 69 | HbS HPFH | BT (1) | SCD pt. requiring ICU admission w/ intubation and mechanical ventilation quickly improved following RBC exchange transfusion. | n/a | 0 (0) |
| Odiévre et al. [85] | France | 1 (0/1) | 16 | HbSS | HU (1) BT (1) |
Tocilizumab proved to be an effective treatment for this SCD patient with severe COVID-19 and ACS. | n/a | 0 (0) |
| Okar et al. [68] | Qatar | 1 (1/0) | 22 | n/a | HU (1) BT (1) |
Due to similarities between ACS and COVID-19 pneumonia, SCD pts. should be closely monitored and offered BT early in the clinical course to avoid clinical deterioration. | n/a | 0 (0) |
| Okar et al. [69] | Qatar | 1 (1/0) | 48 | n/a | HU (1) BT (1) |
Close monitoring of SCD pts. is essential despite favorable outcomes due to acute complications; BT early in the disease course is beneficial against hemolysis and VOC. | n/a | 0 (0) |
| Parodi et al. [86] | Italy | 2 (1/1) | 1.2 | HbSS (2) | BT (2) | Severe SARS-CoV-2 infection revealed a hidden condition of SCD. | n/a | 0 (0) |
| Quaresima et al. [87] | Italy | 1 (0/1) | 18 | HbSS | HU (1) BT (1) |
Pt had ACS and abnormal chest CT but no resp. symptoms, remained COVID-19 pos for a long time, and rare blood type prohibited BT. | n/a | 0 (0) |
| Roussel et al. [88] | France | 1 (0/1) | 6 | HbSS | n/a | Cranial polyneuropathy was the first manifestation of severe COVID-19 in a child and cranial nerve involvement may indicate poor disease course. | n/a | 0 (0) |
| Sahu et al. [45] | USA | 5 (2/3) | 33 | HbSS (2), HbSC (2), HbSβ (1) |
HU (2) BT (1) |
None of the pts. required intensive care or mechanical ventilation with only one patient had a new oxygen requirement. SCD COVID-19 pts. need multidisciplinary treatment of pneumonia, VOC, ACS, with supportive care for favorable outcome. |
4 (80) | 0 (0) |
| Santos de Lima et al. [114] | USA | 1 (0/1) | 43 | n/a | BT (1) | Combination of COVID-19 and SCD may increase risk of posterior reversible encephalopathy syndrome and contribute to higher mortality. | n/a | 0 (0) |
| Teulier et al. [115] | France | 1 (1/0) | 33 | HbSS | HU (1) BT (1) |
SCD patient developed severe ARDS secondary to COVID-19 and was successfully treated with anticoagulation and extracorporeal membrane oxygenation; patient did not develop pulmonary arterial hypertension, suggesting different pathophysiology in SCD pts. | n/a | 0 (0) |
| Walker et al. [89] | USA | 1 (0/1) | 10 | HbSS | BT (1) | Patient developed COVID-19 and ACS requiring red blood cell exchange and remdesivir treatment. | n/a | 0 (0) |
Abbreviations: ACS, acute chest syndrome; ARDS, acute respiratory distress syndrome; BT, blood transfusion; CT, computed tomography; DHTR, delayed hemolytic transfusion reaction; h/o, history of; Hosp., hospitalization; HPFH, hereditary persistent fetal hemoglobin; HU, hydroxyurea; ICU, intensive care unit; n/a, not available, not applicable, unknown, or unspecified; pts., patients; NIV, non-invasive ventilation; PICU, pediatric intensive care unit; SCD, sickle cell disease; VOC, vaso-occlusive crisis.