A report of six TAFRO syndrome: clinical characteristics, diagnosis and treatment analysis

Abstract

Objective: To explore the clinical characteristics, treatment and prognosis of TAFRO syndrome. Methods: All patients diagnosed as Castleman disease in Peking University People's Hospital between December 2011 and April 2019 were included.Among them,6 patients were diagnosed as TAFRO syndrome. Medical records were studied;the clinical manifestation, laboratory test, pathology, treatment and prognosis were analyzed. Recent related literatures were reviewed. Results: The average age of six TAFRO syndrome patients (5 males)was 41.5 years(range, 27-59 years). The patients presented as acute or subacute onset, manifested as fever, thrombocytopenia, polyserositis including pleural effusion and ascites, organomegaly, anasarca, and renal insuffciency. One patient was accompanied by hemophagocyticsyndrome, one patient was accompanied by hypothyroidism, six patients' serum IL-6 was elevated, four patients had received the test of serum VEGF and results were all elevated, six patients' HIV antibody were negative,four patients had received HHV-8 DNA test and results were all negative. For pathology, threewere plasma cell type, twowere mixed type andonewashyaline vascular type. Renal biopsies were performed in 2 patients, showing that renal thrombotic microangiopathyassociated with subacute tubulointerstitial nephritis and secondary capillary proliferative glomerulonephritis. CHOP chemotherapy wereused in 2 patients, glucocorticoid was used in 1 patient, and glucocorticoid combined with Rituximab or Tocilizumab were used in 3 patients. Among them, one patient died because of disease progression after 5 years, other five patientsare still stable. Conclusion: TAFRO syndrome is a rare disease, early recognition and appropriate treatment may improvethe prognosis.