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Table 2 Previously reported cases of TAFRO syndrome required hemodialysis during clinical course and the present case

From: Severe refractory TAFRO syndrome requiring continuous renal replacement therapy complicated with Trichosporon asahii infection in the lungs and myocardial infarction: an autopsy case report and literature review

Case number [Reference] Reported year Age /Sex Underlying disease Immunity anomalies IL-6 (pg/mL) LN histology Durations from admission to initiation of HD Immunosuppressive treatment Complications Renal outcome Clinical outcome
Case 1 [44] 2013 56/M ITP H. pylori infection Platelet-associated anti-GPIIb/IIIa Ab 7.2 (serum) N/A N/A mPSLp, IVIG, CsA N/A Withdrawal from HD (durations, N/A) Alive
Case 2 [44] 2013 56/F N/A None 9.05 (serum) N/A 6 days mPSLp, PSL, CsA CMV infection Withdrawal from HD after 1 month Alive
Case 3 [45] 2013 47/F N/A None 21.9 (serum) PC-type 17 days mPSLp, PSL, TCZ CMV pneumonia Withdrawal from HD after 2 months Alive
Case 4 [28] 2016 47/F None N/A 4380 (serum) 1600 (CSF) N/A 1 day mPSLp, PSL Respiratory failure requiring mechanical ventilation Withdrawal from HD after 3 weeks Alive
Case 5 [28] 2016 76/M None ANA 14.1 (N/A) HV-type 10 days mPSLp, mPSL sodium succinate Sepsis (due to systemic, Staphylococcus aureus and, CMV infections) Withdrawal from HD after about 2 weeks Died
Case 6 [42] 2016 22/M EBV infection N/A N/A Mixed-type N/A (about 2 weeks) TCZ, RTX, ETP Respiratory failure requiring intubation Withdrawal from HD (durations, N/A) Alive
Case 7 [43] 2016 73/M DM Uveitis ANA 5.3 (serum) Biopsy was not performed N/A (about 2 weeks) PSL DVT, DLBCL Withdrawal from HD after 1 week Alive
Case 8 [46] 2016 49/F HTN ANA 83.4 (serum) HV-type 4 days mPSLp, PSL, TCZ Respiratory failure requiring mechanical ventilation, liver failure, hemorrhage from a rectal ulcer, sepsis due to CNS Withdrawal from, CHDF and HD, After 2 months Alive
Case 9 [19] 2016 48/M None ANA, anti-SS-A Ab anti-TPO Ab, positive direct Coombs test 16.8 (serum) 945 (pleural effusion) N/A 42 days mPSLp, PSL, IVIG, PE, RTX Cardiogenic shock due to cardiomyopathy Withdrawal from HD after 2 months Alive
Case 10 [47] 2016 72/M HTN, HL None 46.4 (N/A) Mixed-type 14 days PSL, TCZ Cerebral infarction, necrosis of the ascending colon due to infarction HDF was continued until the patient’s death Died
Case 11 [48] 2016 50/M N/A Decreased ADAMTS13 activity (9.9%) 2130 (N/A) HV-type 9 days mPSLp, PSL, TCZ N/A Withdrawal from HD after 3 months Alive
Case 12 [17], 2017 59/M DU, Fatty liver RF 15.2 (N/A) Biopsy was not performed N/A (about 3 months) mPSLp, PSL, TCZ Bacterial infections (multiple times), PCP, CMV infection, Candida bacteremia Withdrawal from CHDF and HDF after 5 months Alive
Case 13 [20] 2017 72/M N/A Anti-SS-A Ab 26.8 (serum) Not performed N/A (about 2 weeks) mPSLp, PSL, CYA, TCZ Temporary cardiac arrest, hypovolemic shock and respiratory failure, Corynebacterium sepsis and pneumonia Withdrawal from CHDF and HD after 3 months Alive
Case 14 [49] 2017 70/M Esophageal cancer λ-type BJP, slightly decreased ADAMTS13 activity 33 (serum) Not performed 21 days mPSLp, PSL N/A Withdrawal from HD after 1 week Alive
Case 15 [50] 2017 59/M HBV infection None 14 (N/A) Mixed-type 17 days mPSLp, PSL, TCZ, and CsA Cardiac arrest possibly caused by massive ascites Withdrawal from CRRT and HD after 1 month Alive
Case 16 [51] 2017 38/M DU None 26.6 (serum) HV-type 4 days mPSLp, PSL, CsA, and TCZ DIC Withdrawal from CHDF and HD after 1 month Alive
Case 17 [52] 2017 24/F N/A During pregnancy 842 (serum) Biopsy was not performed 8 days mPSLp, PSL Respiratory failure required mechanical ventilation Withdrawal from HD after about 3 weeks Alive
Case 18 [53] 2017 61/F N/A anti-CCP Ab 75.9 (serum) HV-type 7 days mPSLp, TCZ, RTX Multiple organ failure requiring mechanical ventilation Withdrawal from CRRT and HD (durations, N/A) Alive
Case 19 [33] 2018 50/F Pityriasis rosea ANA, anti-SS-A/SS-B Ab, anti-Tg/TPO Ab 15.9 (serum) 5480 (ascites) Mixed-type About 20 months after fist admission mPSLp, PSL, RTX CMV infection ARDS required mechanical ventilation, Septic shock due to cholecystitis Withdrawal from CHDF and HD (durations, N/A) Alive
Case 20 The present case 46/M None ANA, anti-SS-A Ab 25.2 (serum) 3310 (ascites) Mixed-type 50 days mPSLp, PSL, CsA Cardiac arrest due to MI, bacterial peritonitis, systemic CMV infections, Trichosporon asahii infection in the lungs CHDF was continued until the patient’s death Died
  1. Ab antibody, ADAMTS13 A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13, ARDS acute respiratory distress syndrome, ANA antinuclear antibody, BJP Bence Jones protein, CCP cyclic citrullinated peptide, CHDF continuous hemodiafiltration, CNS coagulase-negative staphylococci, CMV cytomegalovirus, CRRT continuous renal replacement therapy, CsA cyclosporine A, CSF cerebrospinal fluid, DIC disseminated intravascular coagulation, DLBCL diffuse large B-cell lymphoma, DU duodenal ulcer, DVT deep vein thrombosis, EBV Epstein-Barr virus, ETP etoposide, F female, GP glycoprotein, HBV hepatitis B virus, HD hemodialysis, H.pylori Helicobacter pylori, HV hyaline vascular, IL-6 interleukin 6, ITP immune thrombocytopenic purpura, IVIG intravenous immunoglobulin, LN lymph node, M male, MI myocardial infarction, mPSLp methyl prednisolone pulse therapy, N/A not available, PC plasma cell, PCP pneumocystis pneumonia, PE plasma exchange, RF rheumatoid factor, RTX rituximab, SS Sjögren syndrome, TCZ tocilizumab, Tg thyroglobulin, TPO thyroperoxidase, VEGF vascular endothelial growth factor