Table 1.
Summary of demographic and clinical characteristics of patients presenting as COVID-19-associated TTP.
| Reference | Sex | Age | Known Comorbidities | Reported Signs/Symptoms Associated with COVID-19 | Proposed Underlying Pathophysiology of TMA (Excluding COVID-19) | Biopsy-Proven TMA | PCR/Serology for SARS-CoV-2 | Kidney Function/Serum Creatinine (μmol/L) | Lowest Platelet Count (×106/L) |
Accelerated Hypertension | APL Antibodies | TMA-Specific Treatment | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Kornowski Cohen [29] | F | 62 | SLE, APLS, CVA, hyperlipidemia trigeminal neuralgia, hypothyroidism | Abdominal pain, mild cough, low-grade fever | ADAMTS13 activity undetectable, borderline anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | 40 at admission, no further data | 41 | No | Not assessed (history of APLS) | TPE, steroids, caplacizumab, rituximab | Recovered, no data on renal function |
| Tehrani [30], Darnahal [31] | F | 56 | History of locally advanced breast cancer on adjuvant therapy | Fever, dyspnea | ADAMTS13 activity 0.01 IU/mL, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | No data | 41 | No | Not assessed | TPE, rituximab | Died—hemorrhagic CVA |
| Tehrani [30] | F | 57 | No comorbidities | Severe respiratory symptoms | ADAMTS13 activity 0,86 IU/mL, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | No data | 98 | No | Not assessed | Infusion of FFP (shortage of TPE setting), IVIG | Recovered, no data on renal function |
| Tehrani [30] | M | 38 | No comorbidities mentioned | No respiratory symptoms, rectorrhagia | ADAMTS13 activity 0,06 IU/mL, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | No data | 5 | No | Not assessed | TPE, rituximab | Recovered, no data on renal function |
| Tehrani [30] | F | 25 | Third-trimester pregnancy | Fever, dyspnea | ADAMTS13 activity 8%, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | No data | 10 | No | Not assessed | TPE, preterm delivery induction | Recovered, no data on renal function |
| Nicolotti [32] | F | 44 | Obesity, history of deep vein thrombosis | Weakness, dizziness, abdominal discomfort | ADAMTS13 activity <5%, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | 203 at admission, normal after treatment (no specific data) | 7 | No | Not assessed | TPE, steroids, rituximab, caplacizumab | Complete recovery of renal function |
| Albiol [33] | F | 57 | Arterial hypertension, breast cancer in complete remission | Fever, dry cough, anosmia, dysgeusia | ADAMTS13 activity 2%, + anti-ADAMTS13 antibodies | Not performed | − PCR, + IgG | 70 at admission, 53 after treatment | 13 | No | Not assessed | Steroids, TPE | Complete recovery of renal function |
| Hindilerden [34] | F | 74 | Arterial hypertension | Fatigue, cough | ADAMTS13 activity 0.2%, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | No data | 48 | No | Not assessed | TPE, steroids | Recovered, no data on renal function |
| Maharaj [35] | F | 69 | TTP with multiple relapses, status post PE and cerebral infarct | Acute hypoxic respiratory failure | ADAMTS13 activity 2.9%, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | No data | 100 | No | Not assessed | TPE, steroids | Died |
| Capecchi [36] | F | 55 | Secondary TTP due to bacterial pneumonia 30 years ago | Mild influenza-like symptoms 1 month prior to current admission | ADAMTS13 activity undetectable, + anti-ADAMTS13 antibodies | Not performed | − PCR, + IgG | 281 at admission, 59 at discharge | 14 | No | Not assessed | TPE, steroids, caplacizumab | Complete recovery of renal function |
| Dhingra [37] | F | 35 | No comorbidities | Loose stools 15 days prior to current admission | ADAMTS13 activity undetectable, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | 40–80 (66 at admission, 58 at discharge) | 20 | No | Negative | TPE, steroids, rituximab | Recovered, intact renal function from the start |
| Shankar [38] | M | 30 | No comorbidities, obesity | No respiratory symptoms, back pain, hematuria | ADAMTS13 activity 3%, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | 134 at admission, 68 at discharge | 4 | No | Not assessed | TPE, steroids, caplacizumab | Complete recovery of renal function |
| Beaulieu [39] | M | 70 | Peripheral artery disease, dyslipidemia | Asymptomatic | ADAMTS13 activity <10%, + anti-ADAMTS13 antibodies (low titer) | Not performed | + PCR, serology not done | 106 at admission, 67 at discharge | 18 | No | Not assessed | Steroids, TPE | Intact renal function, complete neurological recovery |
| El-Sawalhy [40] | M | 49 | Arterial hypertension, hyperlipidemia | Left-lower-quadrant abdominal tenderness | ADAMTS13 activity <5% | Not performed | + (test not specified) | No data | 12 | No | Not assessed | TPE, steroids | Recovered, no data on renal function |
| Alhomoud [41] | M | 62 | Crohn’s disease, G6PD deficiency, TTP | Shortness of breath, generalized weakness, chills | ADAMTS13 activity <5%, − anti-ADAMTS13 antibodies | Not performed | not specified | 265 at admission, normal after treatment (no specific data) | 21 | No | Not assessed | TPE, steroids, rituximab | Complete recovery of renal function |
| Law [42] | F | 47 | No comorbidities | Progressive fatigue | ADAMTS13 activity <5%, + anti-ADAMTS13 antibodies | Not performed | + PCR, serology not done | 71 at admission, 56 4 days before discharge | 6 | No | Negative | TPE, caplacizumab | Complete recovery of renal function |
| Altowyan [43] | M | 39 | Diabetes mellitus type 2 | Nausea, vomiting, no respiratory symptoms | PLASMIC score 6 (ADAMTS13 activity not assessed) | Not performed | + PCR, serology not done | 77 at admission, no further data | 6 | No | Negative | TPE, steroids, rituximab | Recovered, renal function intact |
| Verma [44] | M | 21 | No comorbidities | Fever, dyspnea, cough | PLASMIC score 7 (ADAMTS13 activity not assessed) | Not performed | + PCR, serology not done | 61 during hospitalization (not further specified) | 10 | No | Not assessed | FFP (TPE withheld—severe hypotension) | Died (sepsis, progressive TTP) |
Legend: APL, antiphospholipid; APLS, antiphospholipid syndrome; COVID-19, coronavirus disease 2019; CVA, cerebrovascular accident; FFP, fresh frozen plasma; G6PD, glucose-6-phosphate dehydrogenase; IVIG, intravenous immunoglobulin; PCR, polymerase chain reaction; SLE, systemic lupus erythematosus; TMA, thrombotic microangiopathy; TPE, therapeutic plasma exchange; TTP, thrombotic thrombocytopenic purpura.