Table 1.
Results of the 32 Cases, Published During the 2020–2022 Period, Including Thrombotic Thrombocytopenic Purpura (TTP) following COVID-19 Vaccination.
| Authors and Ref | Country (Year) |
Old Gender | Underlying Disease | First Episode | Symptoms | Vaccine | Biology | ADAMTS 13 Activity | Treatments | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|
| Relapse | Dose | Autoantibody * | ||||||||
| Time after Vaccination | ||||||||||
| Chamarti et al. [20] | USA (2021) |
80 | Hypertension | First | Generalized weakness | Pfizer- BioNTech |
Hemoglobin, 4.8 g/dL | <2% | Plasma Exchange Steroids | Improved |
| Male | Diabetes | Malaise | Second dose | Platelets, 48 × 109/L | 182 U/mL | Rituximab | ||||
| Hyperlipidemia | 14 days | Schistocytes, +++ | ||||||||
| Gout | Creatinine, 212.16 µmol/L | |||||||||
| Iron deficiency | LDH, 1118 UI/L | |||||||||
| Anemia | Haptoglobin, <10 mg/dL | |||||||||
| Giuffrida et al. [14] | Italy (2021) |
83 | Undifferentiated connective tissue disease | First | Severe anemia | Pfizer- BioNTech |
Hemoglobin, 6.1 g/dL | <10% | Plasma Exchange Steroids | Death (probably due to a |
| Female | Diabetes | Macro-hematuria | First dose | Retic, 28% | 40 U/mL | Caplacizumab | sudden cardiovascular event) | |||
| Diffuse petechiae | 7 days | Platelets, 46 × 109/L | ||||||||
| Schistocytes, 10% | ||||||||||
| Creatinine, 77.79 µmol/L | ||||||||||
| LDH, 1905 UI/L | ||||||||||
| Haptoglobin, <7 mg/dL | ||||||||||
| 30 | Beta-thalassemia | First | Diffuse petechiae | Pfizer- BioNTech |
Hemoglobin, 8.9 g/dL | <10% | Plasma Exchange Steroids | Improved | ||
| Female | Intense headache | First dose | Retic, 29% | 77.6 U/mL | Caplacizumab | |||||
| Fatigue | 18 days | Platelets, 11 × 109/L | ||||||||
| Schistocytes, 5–10% | ||||||||||
| Creatinine, 79.56 µmol/L | ||||||||||
| LDH, 900 UI/L | ||||||||||
| Haptoglobin, <7 mg/dL | ||||||||||
| Karabulut et al. [11] | USA (2021) |
48 | No | First | Acute-onset, transient right-sided weakness | Moderna Biotech | Hemoglobin, 8.8 g/dL | <3% | Plasma Exchange Steroids | Improved |
| Male | Slurred speech lasting | First dose | Platelets, 10 × 109/L | 6.6 BEU | Rituximab | |||||
| 5 days | Schistocytes, 2–3% | |||||||||
| Creatinine, 83.98 µmol/L | ||||||||||
| LDH, 884 UI/L | ||||||||||
| Haptoglobin, <10 mg/dL | ||||||||||
| Lee et al. [28] | UK (2021) | 50 | Hypertension | First | Dysphasia | AstraZeneca | Hemoglobin, 9.9 g/dL | 0% | Plasma Exchange Steroids | Improved |
| Female | Acute upper limb numbness | First dose | Retic, 6.9% | 94.93 U/mL | Rituximab | |||||
| 12 days | Platelets, 33 × 109/L | |||||||||
| Schistocytes, + | ||||||||||
| LDH, 359 UI/L | ||||||||||
| Maayan et al. [29] | Israel (2021) |
40 | No | First | Somnolence | Pfizer- BioNTech |
Hemoglobin, 9.9 g/dL | 0% | Plasma Exchange | Improved |
| Female | Fever | Second dose | Platelets, 12 × 109/L | 51 U/mL | Steroids | |||||
| Macroscopic hematuria | 8 days | Schistocytes, 6% | Caplacizumab | |||||||
| Creatinine, 81.35 µmol/L | ||||||||||
| LDH, 7129 UI/L | ||||||||||
| 28 | Morbid obesity | First | Dysarthria | Pfizer- BioNTech |
Hemoglobin, 9.1 g/dL | 0% | Plasma Exchange Steroids | Improved | ||
| Male | Second dose | Platelets, 38 × 109/L | 113 U/mL | Caplacizumab Rituximab | ||||||
| 28 days | Schistocytes, 6% | |||||||||
| Creatinine, 132.63 µmol/L | ||||||||||
| LDH, 3063 UI/L | ||||||||||
| 31 | TTP | Relapse | Vaginal bleeding | Pfizer- BioNTech |
Hemoglobin, 7.7 g/dL | 0% | Plasma Exchange Steroids | Continu caplacizumab | ||
| Female | Purpura | First dose | Platelets, 17 × 109/L | 64 U/mL | Caplacizumab | |||||
| 13 days | Schistocytes, 10% | Rituximab | ||||||||
| Creatinine, 106 µmol/L | ||||||||||
| LDH, 4000 UI/L | ||||||||||
| 30 | TTP | Relapse | Purpura | Pfizer- BioNTech |
Hemoglobin, 8.3 g/dL | 0% | Plasma Exchange Steroids | Improved | ||
| Male | Second dose | Retic, 8% | 21 U/mL | Caplacizumab | ||||||
| 8 days | Platelets, 14 × 109/L | Rituximab | ||||||||
| Schistocytes, 14% | ||||||||||
| Renal function, normal | ||||||||||
| LDH, 1138 UI/L | ||||||||||
| Osmanodja et al. [35] | Germany (2021) |
25 | No | First | Persisting malaise | Moderna Biotech | Hemoglobin, 7.4 g/dL | <5% | Plasma Exchange Steroids | Continu caplacizumab |
| Male | Fever | First dose | Retic, 233.1 109/L | 72.2 U/ml | Caplacizumab | |||||
| Headache | 13 days | Platelets, 29 × 109/L | Rituximab | |||||||
| Word-finding difficulties | Schistocytes, 2.1% | |||||||||
| Nausea, vomiting | Creatinine, 132.6 µmol/L | |||||||||
| Petechial bleeding | LDH, 999 UI/L | |||||||||
| Hematuria | Haptoglobin, <8 mg/dL | |||||||||
| Pavenski et al. [30] | Canada (2021) |
84 | TTP | Relapse | Lethargy | Pfizer- BioNTech |
Hemoglobin, 7.2 g/dL | <1% | Plasma Exchange | Improved |
| Male | Prostate cancer Hypertension Diabetes | Myalgias | First dose | Retic, elevated | >15 U/mL | Steroids | ||||
| Gout | Anorexia | 7 days | Platelets, 58 × 109/L | Rituximab | ||||||
| Hypercholesterolemia | Schistocytes, + | |||||||||
| Creatinine, 77 µmol/L | ||||||||||
| LDH, 594 UI/L | ||||||||||
| Sissa et al. [36] | Italy (2021) | 48 | TTP | Relapse | Ecchymosis | Pfizer- BioNTech |
Hemoglobin, 11.5 g/dL | <3% | Plasma Exchange | Improved |
| Female | Second dose | Platelets, 94 × 109/L | 88 U/mL | Steroids | ||||||
| 6 days | Schistocytes, 10% | |||||||||
| Renal function, normal | ||||||||||
| LDH, 637 UI/L | ||||||||||
| Waqar et al. [22] | USA (2021) |
69 | Hypertension Chronic kidney disease | First | Severe fatigue | Pfizer- BioNTech |
Hemoglobin, 9.3 g/dL | 2% | Plasma Exchange Steroids | Improved |
| Male | HIV | Shortness of breath | Second dose | Retic, 2.8% | >90 U/mL | Rituximab | ||||
| Chronic hepatitis B | 7 days | Platelets, 22 × 109/L | ||||||||
| Deep | Schistocytes, ++ | |||||||||
| vein thrombosis | Creatinine, 177.68 µmol/L | |||||||||
| LDH, 1229 UI/L | ||||||||||
| Yucum et al. [37] | USA (2021) |
62 | Hypertension | first | Acute onset of altered mental status | Johnson and Johnson | Hemoglobin, 8.2 g/dL | <12% | Plasma Exchange Steroids | Improved |
| Female | Hyperlipidemia | First dose | Retic, 8% | NA | Hemodialysis | |||||
| Hypothyroidism | 37 days | Platelets, 11 × 109/L | ||||||||
| Creatinine, 530 µmol/L | ||||||||||
| LDH, >2500 UI/L | ||||||||||
| ASAT/ALAT, 982/231 U/L | ||||||||||
| Al Ahmad et al. [21] | Kuwait (2021) |
37 | Secondary polycythemia | first | Dizziness, fatigue | AstraZeneca-Oxford | Hemoglobin, 8.3 g/dL | 2.60% | Plasma Exchange Steroids | Improved |
| Male | Headache | First dose | Retic, 8% | Positive | Rituximab | |||||
| Shortness of breath | 10 days | Platelets, 14 × 109/L | ||||||||
| Palpitation | Schistocytes, 14% | |||||||||
| Dark urine and petechiae | Renal function, normal | |||||||||
| LDH, 1138 UI/L | ||||||||||
| De Bruijn et al. [25] | Belgium (2021) |
38 | No | First | Spontaneous | Pfizer- BioNTech |
Hemoglobin, 10.5 g/dL | 0% | Plasma Exchange | Improved |
| Female | bruising and petechiae | First dose | Retic, 263 109/L | 106.8 BEU | Steroids | |||||
| 14 days | Platelets, 46 × 109/L | Caplacizumab | ||||||||
| Schistocytes, 3% | Rituximab | |||||||||
| Creatinine, 83.98 µmol/L | ||||||||||
| LDH, 631 UI/L | ||||||||||
| Alislambouli et al. [12] | USA (2022) |
61 | No | First | Confusion | Pfizer- BioNTech |
Hemoglobin, 6.5 g/dL | <3% | Plasma Exchange | Improved |
| Male | Fever | First dose | Retic, 8% | NA | Steroids | |||||
| Headache | 5 days | Platelets, 6 × 109/L | Rituximab | |||||||
| Emesis | Schistocytes, 8% | |||||||||
| Dark urine | LDH, 1757 UI/L | |||||||||
| Leg ecchymosis | Haptoglobin, <8 mg/dL | |||||||||
| Deucher et al. [38] | USA (2022) |
28 | TTP | Relapse | Bruising on arms | Pfizer- BioNTech |
Hemoglobin, 10.5 g/dL | <2.5% | Caplacizumab | Improved |
| Female | First dose | Platelets, 84 × 109/L | Positive | Steroids | ||||||
| 5 days | Schistocytes, ++ | Rituximab | ||||||||
| LDH, 205 UI/L | ||||||||||
| Haptoglobin, undetectable | ||||||||||
| Innao et al. [26] | Italy (2022) |
33 | Hodgkin Lymphoma | First | Asthenia | Pfizer- BioNTech |
Hemoglobin, 6.8 g/dL | 8% | Plasma Exchange | Improved |
| Female | Gray Zone Lymphoma | Drowsiness | First dose | Retic, 896 × 109/L | 5 U/mL (not valuable due to defects in the sample) | Steroids | ||||
| Headache | 9 days | Platelets, 12 × 109/L | Caplacizumab | |||||||
| Nausea | Schistocytes, 3% | |||||||||
| Abdominal pain | Creatinine, 122 µmol/L | |||||||||
| Lower extremity purpura | LDH, 1280 UI/L | |||||||||
| Haptoglobin, <6 mg/dL | ||||||||||
| Kirpalani et al. [39] | Japan (2022) |
14 | Anxiety | First | Fatigue | Pfizer- BioNTech |
Hemoglobin, 6.3 g/dL | <1% | Plasma Exchange Steroids | Improved |
| Female | Iron | Headache | First dose | Platelets, 10 × 109/L | 72 U/mL | Caplacizumab | ||||
| Deficiency | Confusion | 14 days | Schistocytes, + | Rituximab | ||||||
| Bruising | LDH, 626 UI/L | |||||||||
| Haptoglobin, <10 mg/dL | ||||||||||
| Ruhe et al. [40] | Germany (2022) | 84 | No | First | Partial hemiplegia | Pfizer- BioNTech |
Hemoglobin, 7.9 g/dL | 1.60% | Plasma Exchange Steroids | Improved |
| Female | Scattered petechiae | First dose | Platelets, 45 × 109/L | 82.2 U/mL | Rituximab | |||||
| 16 days | Schistocytes, 4.2% | |||||||||
| Creatinine, 172.38 µmol/L | ||||||||||
| Haptoglobin, <10 mg/dL | ||||||||||
| Yoshida et al. [13] | Japan (2022) |
57 | Acute hepatitis of unknown cause | First | Fatigue | Pfizer- BioNTech |
Hemoglobin, 5.5 g/dL | <0.5% | Plasma Exchange Steroids | Improved |
| Male | Loss of appetite | First dose | Retic, 496 × 109/L | 1.9 BU/mL | Rituximab | |||||
| Jaundice | 7 days | Platelets, 9 × 109/L | ||||||||
| Schistocytes, 17.6% | ||||||||||
| Creatinine, 138.87 µmol/L | ||||||||||
| LDH, 2275 UI/L | ||||||||||
| Haptoglobin, 3 mg/dL | ||||||||||
| Picod et al. [41] | France (2022) |
36 | Systemic lupus erythematosus | First | Bruising | Pfizer- BioNTech |
Hemoglobin, 10 g/dL | <5% | Plasma Exchange Steroids | Improved |
| Female | Headache | First dose | Platelets, 10 × 109/L | 0.5 BU/mL | Rituximab | |||||
| 6 days | Schistocytes, 3% | |||||||||
| Creatinine, 86.24 µmol/L | ||||||||||
| 54 | TTP | Relapse | Bruising | Moderna Biotech |
Hemoglobin, 11.5 g/dL | <5% | Plasma Exchange Steroids | Improved | ||
| Male | Diffuse | First dose | Platelets, 17 × 109/L | 1.1 BU/mL | Rituximab Caplacizumab | |||||
| mucocutaneous | First dose | Schistocytes, 2% | ||||||||
| bleeding | 23 days | Creatinine, 149.6 µmol/L | ||||||||
| Headache | ||||||||||
| Amnesia | ||||||||||
| 60 | TTP | Relapse | Cerebellar | Pfizer- BioNTech |
Hemoglobin, 10.8 g/dL | <10% | Plasma Exchange Steroids | Improved | ||
| Female | Syndrome | First dose | Platelets, 27 × 109/L | Positive | Rituximab | |||||
| 10 days | Schistocytes, 2% | |||||||||
| Creatinine, 66.88 µmol/L | ||||||||||
| 60 | No | First | Cerebellar | Pfizer- BioNTech |
Hemoglobin, 6.5 g/dL | 5% | Plasma Exchange Steroids | Improved | ||
| Female | Syndrome | First dose | Platelets, 20 × 109/L | 52 U/mL | Caplacizumab | |||||
| Aphasia | 12 days | Schistocytes, 6% | ||||||||
| Confusion | Creatinine, 80.96 µmol/L | |||||||||
| Chest pain | ||||||||||
| 38 | No | First | Fever | Pfizer- BioNTech |
Hemoglobin, 6.6 g/dL | <1% | Plasma Exchange Steroids | Improved | ||
| Male | Headache | Second dose | Platelets, 9 × 109/L | Positive | Rituximab Caplacizumab | |||||
| Hemiparesis | 30 days | Schistocytes, 5% | ||||||||
| Bruising | Creatinine, 88.88 µmol/L | |||||||||
| 68 | Mixed connective tissue disease | Relapse | Dizziness | Pfizer- BioNTech |
Hemoglobin, 10.9 g/dL | 2% | Plasma Exchange Steroids | Improved | ||
| Male | TTP | First dose | Platelets, 39 × 109/L | - | Rituximab Caplacizumab | |||||
| 17 days | Schistocytes, 1% | |||||||||
| Creatinine, 69.52 µmol/L | ||||||||||
| 66 | No | First | Facial paralysis | AstraZeneca-Oxford | Hemoglobin, 7.9 g/dL | <5% | Plasma Exchange | Improved | ||
| Male | First dose | Platelets, 11 × 109/L | - | Steroids | ||||||
| 8 days | Schistocytes, 4% | Rituximab | ||||||||
| Creatinine, 81.84 µmol/L | Caplacizumab | |||||||||
| 70 | Ischemic strokes | First | Coma | AstraZeneca-Oxford | Hemoglobin, 8 g/dL | 11% | Intravenous Immunoglobulins Plasma Infusion Steroids | Death 2 month | ||
| Female | Hypertension | Hemiparesis | First dose | Platelets, 6 × 109/L | 140 U/mL | Rituximab Caplacizumab | after presentation | |||
| 10 days | Schistocytes, 2% | |||||||||
| Creatinine, 79.2 µmol/L | ||||||||||
| 22 | No | First | Coma | Pfizer- BioNTech |
Hemoglobin, 6.8 g/dL | 6% | Plasma Exchange Steroids | Improved | ||
| Male | Seizures | Second dose | Platelets, 10 × 109/L | Positive | Rituximab | |||||
| Purpura | 18 days | Schistocytes, 2% | ||||||||
| Fever | Creatinine, 101.2 µmol/L | |||||||||
| 20 | Systemic lupus erythematosus | First | Systemic lupus erythematosus Flare | Pfizer- BioNTech |
Hemoglobin, 5.3 g/dL | <10% | Plasma Infusion Steroids | Improved | ||
| Female | Polyarthritis | First dose | Platelets, 51 × 109/L | 50 U/mL | ||||||
| Erythema | 25 days | Schistocytes, 3% | ||||||||
| Creatinine, 88 µmol/L |
* Autoantibodies to ADAMTS-13 was assessed either as the titer of total autoantibodies with a simplified enzyme-linked immunosorbent assay (ELISA) and expressed in arbitrary units (U/mL; normal < 12 U/mL) or as the titer of inhibitory antibodies using an alternative methodology (Bethesda assay) expressed in Bethesda Units (BU/mL; normal < 1 BU/mL) or BEU (normal < 0.4). NA: Not available. Retic, reticulocytes; LDH, Lactate dehydrogenase; +++, semi-quantitative appreciation of schistocytes.