Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan

Fujimoto, Shino; Kawabata, Hiroshi; Sakai, Tomoyuki; Yanagisawa, Hiroto; Nishikori, Momoko; Nara, Kenji; Ohara, Shuichi; Tsukamoto, Norifumi; Kurose, Nozomu; Yamada, Sohsuke; Takai, Kazue; Aoki, Shin; Yasukawa, Masaki

doi:10.1007/s12185-020-03008-3

Cited by 25 publications

(47 citation statements)

References 18 publications

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“…We divided these patients into groups according to the second-line treatments (rituximab, tocilizumab, and cyclosporine A groups) and compared their efficacies based on the primary endpoint set as the time-to-next-treatment or death (TTNT). In this previous study, we found that the TTNT of patients who received rituximab was significantly longer than that of patients who received tocilizumab, although the inter-group differences in overall survival (OS) were not significant [18]. In this cohort, 15 patients who received corticosteroids alone survived beyond 1 year without receiving other immunosuppressive agents.…”

Section: Introductionmentioning

confidence: 64%

“…International consensus treatment guidelines recommend interleukin 6 (IL-6) blockade (siltuximab or tocilizumab) with or without high-dose steroids as the first-line treatment for iMCD-TAFRO [16], but this recommendation was primarily based on a clinical trial that was conducted for entire iMCD patients; the number of enrolled iMCD-TAFRO patients is undescribed [17]. To find an optimal treatment strategy for TAFRO syndrome, we recently analyzed the efficacies of immunosuppressive agents by utilizing a patient registry in Japan [18]. Among 81 patients with TAFRO syndrome, 68 patients received corticosteroids as the first-line treatment, and 47 patients received additional immunosuppressive agents as the second-line treatments.…”

Section: Introductionmentioning

confidence: 99%

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Patient’s age and d-dimer levels predict the prognosis in patients with TAFRO syndrome

et al. 2021

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To identify prognostic factors for TAFRO syndrome, a rare inflammatory disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. Data of patients with TAFRO syndrome were extracted from a Japanese patient registry. Patients were divided into groups according to the clinical and laboratory parameters at initial presentation. Cut-off values for the laboratory parameters were determined using receiver operating characteristic curve analysis and by clinical relevance. Patient survival was analyzed by the Kaplan-Meier method. Univariable analysis was performed using log-rank tests. Multivariable analyses were performed with the logistic regression model and the Cox-proportional hazards model. We extracted the data of 83 patients with TAFRO syndrome from the registry. Univariable analysis identified several potential prognostic factors. Of these factors, age ≥ 60 years and D-dimer ≥ 18 μg/dL remained significant predictors of poor overall survival in the multivariable Cox-proportional hazards model. Based on these results, we developed a simple prognostic scoring system for TAFRO syndrome (TS-PSS). Patients in our cohort were stratified into low, intermediate, and high-risk groups by the TS-PSS. This system should be verified with independent patient cohorts in future studies.

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Section: Introductionmentioning

confidence: 64%

Section: Introductionmentioning

confidence: 99%

Patient’s age and d-dimer levels predict the prognosis in patients with TAFRO syndrome

et al. 2021

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“…In fact, patients with TAFRO syndrome have also been shown to exhibit elevated serum levels of both IL-6 and VEGF [ 1 , 2 , 16 ]. Tocilizumab, an anti-IL-6 receptor antibody, has been widely used as a second-line treatment option for corticosteroid-resistant TAFRO syndrome [ 5 , 17 , 18 , 19 , 20 ]. Meanwhile, previous studies have also reported that patients with TAFRO syndrome who had normal serum IL-6 levels exhibited an inadequate response to tocilizumab [ 5 , 10 , 18 , 19 , 20 ].…”

Section: Discussionmentioning

confidence: 99%

“…Tocilizumab, an anti-IL-6 receptor antibody, has been widely used as a second-line treatment option for corticosteroid-resistant TAFRO syndrome [ 5 , 17 , 18 , 19 , 20 ]. Meanwhile, previous studies have also reported that patients with TAFRO syndrome who had normal serum IL-6 levels exhibited an inadequate response to tocilizumab [ 5 , 10 , 18 , 19 , 20 ]. In the present case, laboratory data before second-line treatment showed increased VEGF levels but normal IL-6 levels.…”

Section: Discussionmentioning

confidence: 99%

Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab

et al. 2021

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TAFRO syndrome, a rare subtype of idiopathic multicentric Castleman disease, manifests as thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Thrombotic microangiopathy, including renal dysfunction, is frequently associated with this syndrome. TAFRO syndrome can be life threatening and show rapid progression, and the diagnosis and management of this disorder remain challenging. A 48-year-old woman was diagnosed with TAFRO syndrome complicated by thrombotic microangiopathy based on the clinical and histopathological findings. After receiving high-dose steroids, her thrombocytopenia and anasarca did not improve. The patient subsequently received a combination of cyclosporine A and rituximab as second-line therapy, which resulted in a significant gradual improvement in the clinical symptoms. Meanwhile, her platelet count increased to more than 40 × 109/L; however, she developed intracranial hemorrhage. Following surgical evacuation, the patient recovered with an achievement of sustained remission. Based on these findings, attention should be paid to life-threatening bleeding associated with local thrombotic microangiopathy even when intensive treatment is administered for TAFRO syndrome.

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“… 6 It was recently reported that patients with TAFRO syndrome exhibit a more aggressive clinical course and greater refractoriness to corticosteroids than those with iMCD-NOS. 7 , 8 Furthermore, they have thrombocytopenia, increased levels of alkaline phosphatase (ALP), and normal levels of gammaglobulin. These clinical manifestations suggested that TAFRO-iMCD and iMCD-NOS should be considered as distinct clinical entities.…”

Section: Introductionmentioning

confidence: 99%

Fatal case of TAFRO syndrome with unilateral adrenal hemorrhage in early-stage disease

Okamoto

Ochi

Motokawa³

et al. 2021

J Clin Exp Hematopathol

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Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly comprise TAFRO syndrome, which was proposed as a distinct clinical entity from iMCD without TAFRO syndrome (iMCD-NOS) due to its aggressive clinical course, refractoriness to corticosteroids, presence of thrombocytopenia, increased level of alkaline phosphatase, and normal level of gammaglobulin. However, diagnosing TAFRO syndrome in its early stages is challenging because it is rare and its diagnostic criteria are complicated. We describe a patient with TAFRO syndrome and adrenal hemorrhage who demonstrated a rapid decline in her clinical condition and did not respond to steroid pulse therapy, resulting in a fatal outcome. In the early stage of her clinical course, she developed unilateral adrenal hemorrhage with mild thrombocytopenia and normal clotting times, suggesting adrenal hemorrhage as a unique manifestation of TAFRO syndrome. In general, patients with TAFRO syndrome exhibit a more aggressive clinical course and poorer outcome than those with iMCD-NOS. To ameliorate this poor prognosis, it is important to diagnose the disease early and immediately start powerful immunosuppressive agents such as tocilizumab. Based on this case, adrenal hemorrhage may suggest TAFRO syndrome, and facilitate the rapid diagnosis of this complicated and rare disease.

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Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan

Cited by 25 publications

References 18 publications

Patient’s age and d-dimer levels predict the prognosis in patients with TAFRO syndrome

Patient’s age and d-dimer levels predict the prognosis in patients with TAFRO syndrome

Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab

Fatal case of TAFRO syndrome with unilateral adrenal hemorrhage in early-stage disease

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