Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging

Abstract

Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria. Our cases showed high serum procalcitonin levels, suggesting bacterial infection as an onset trigger. In addition, Case 1 is the first case complicated with adrenal hemorrhaging. Case 2 is the second case of tocilizumab-resistant TAFRO syndrome successfully treated with rituximab.

Hepatic Campylobacter jejuni infection in patients with Castleman‐Kojima disease (idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome)

“…The absence of interferon γ elevation suggests the absence of viral infection and the possibility of autoinflammation or a bacterial infection . In addition, serum procalcitonin levels were elevated in patient 3 with iMCD‐TAFRO, consistent with previous case reports; this finding is also often closely associated with bacterial infection …”

“…19 The absence of interferon γ elevation suggests the absence of viral infection and the possibility of autoinflammation or a bacterial infection. 20 In addition, serum procalcitonin levels were elevated in patient 3 with iMCD-TAFRO, consistent with previous case reports 19,21 ; this finding is also often closely associated with bacterial infection. 22 In this study, we detected DNA sequences consistent with C. jejuni in liver specimens from the three patients with iMCD-TAFRO and from two patients with inflammatory conditions.…”

A pediatric case of tocilizumab‐resistant TAFRO syndrome treated successfully with rituximab

“…3 In five of seven cases with tocilizumab resistance, rituximab was selected as an additional treatment (Supplemental Table S2). Three cases, including our case achieved complete remission, 18,19 and the other two cases underwent remission with further treatments such as cyclosporine or etoposide in addition to rituximab. 15,17 As a result, rituximab and tocilizumab combined therapy appears to be useful for TAFRO syndrome with tocilizumab resistance.…”

“…13 So far, there are only six cases with apparent resistance or relapse under long-term treatment with anti-IL-6 agents (Supplemental Table S2). [14][15][16][17][18][19] Japanese research group did not clearly state the second-line treatment for refractory TAFRO syndrome. 3 In five of seven cases with tocilizumab resistance, rituximab was selected as an additional treatment (Supplemental Table S2).…”

The first case of thrombocytopenia, anasarca, fever, renal impairment or reticulin fibrosis, and organomegaly (TAFRO) syndrome with unilateral adrenal necrosis: a case report

“…In terms of organ damage, the kidneys are commonly involved, and temporal hemodialysis is required in some cases [ 2 ]. Notably, there have only been a few cases of TAFRO syndrome complicated with adrenal lesions, and all of them have had hemorrhagic involvement [ 4 , 5 ]. No cases accompanied by adrenal gland necrosis without hemorrhage have yet been reported.…”