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

Abstract

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.

Characteristics of thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly syndrome: a retrospective study from a large Western cohort

“…However, contrasting with previous data, Western TAFRO syndrome did not appear to be associated with treatment failure. Moreover, the two-year OS was 96% in the TAFRO group and 97% in the iMCD group, a finding which differed from previously published series (around 65% in the cohort from Fujimoto's et al 5 and 67Á8% in the Multicenter Collaborative Retrospective Study for Establishing the Concept of TAFRO syndrome registry 11 ). Only two deaths were reported, and a complete response was obtained in the majority (91%) of TAFRO patients, with a low relapse rate.…”

“…The median age at diagnosis appeared to be younger in our cohort than in the previously reported Japanese cohorts (Table SIII). 5,6,8–10 This difference may explain the better OS of our TAFRO syndrome patients compared to that of the Japanese cohorts, since age appears to be the strongest predictor of death in a recent report 11 . This difference in age between Western and Eastern cohorts has been already reported in a literature review by Coutier et al 12 …”

“…5,6,[8][9][10] This difference may explain the better OS of our TAFRO syndrome patients compared to that of the Japanese cohorts, since age appears to be the strongest predictor of death in a recent report. 11 This difference in age between Western and Eastern cohorts has been already reported in a literature review by Coutier et al 12 Because of the criteria used to define TAFRO syndrome by the All Japan TAFRO Syndrome Research Group, the TAFRO group unsurprisingly showed a higher prevalence of reticulin fibrosis and kidney failure, a lower gammaglobulin level and a higher alkaline phosphatase level than the iMCD-NOS group. 6,7 Median time from first symptoms to diagnosis was seven weeks and was significantly inferior to the diagnosis delay observed in iMCD-NOS.…”

Castleman disease and TAFRO syndrome

“…This led to the development of a prognostic scoring system for TAFRO syndrome (TS-PSS), with patients in the study cohort stratified into those at low, intermediate, and high risk for TAFRO syndrome. Further studies in independent patient cohorts are needed to verify the prognostic accuracy of the TS-PSS [ 16 ].…”

Thrombocytopenia, anasarca, and severe inflammation

“…Evidence of mammalian target of rapamycin (mTOR) and Janus kinase‐signal transducer and activator of transcription‐3 (JAK‐STAT3) activation has been found in iMCD which provides rationale for investigation of mTOR and JAK inhibitors in this disease 11,19–22 . Age greater than 60 and D‐dimer ≥18 000 μg/L are poor prognostic markers 23 …”