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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