Abstract Number: PB0755
Meeting: ISTH 2021 Congress
Background: TAFRO syndrome as a rare subtype of multi-center Castleman disease is characterized by Thrombocytopenia, Anasarca, myelofibrosis, Renal dysfunction, Organomegaly, lymphadenopathy and a histopathological pattern of atypical Castleman’s disease. It is always fatal if not managed appropriately.
Aims: To present 6 cases of TAFRO syndrome and describe their clinical characteristics and our management experience for the disease.
Methods: 6 patients were admitted during June, 2016 and July, 2020. 3 were male and 3 were female. The age ranged from 42 to 65 years old. All present with high fever unresponsive to antibiotics therapy, lymphadenopathy and splenomegaly. 2 cases had a history of multi-center Castleman disease respectively 2 to 3 years before and were admitted due to recurrence. The other 4 cases received lymph node biopsy and were pathologically diagnosed Castleman disease after admission. All had progressive thrombocytopenia, renal and liver insufficiency, pleural effusion, ascites or pericardial effusion. Bone marrow cellularity were hyperproliferative.
Results: One patient relapsed after CHOP regimen chemotherapy 3 years before received cyclosporin A and tocilizumab treatment. Another one patient only received COP regimen chemotherapy. Four patients received COP regimen chemotherapy due to poor constitutional condition at presentation and then RCHOP regimen after improved condition. 3 patients received cyclosporin A maintenance therapy after chemotherapy. All were responsive to the treatment. Five patients had sustained response and in complete remission. One lost follow-up after one course of therapy.
Conclusions: TAFRO syndrome is always misdiagnosed and may be the result of delayed treatment for multi-center Castleman disease. Careful physical examination to look for lymphadenopathy and lymph node biopsy pathologic analysis is important for timely diagnosis and management. Most of the cases are responsive to rituximab based combined chemotherapy. Cyclosporin A and tocilizumab may be treatment of choice for some patients.
To cite this abstract in AMA style:Ye X. Characteristics and Management of a Series of TAFRO Syndrome [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/characteristics-and-management-of-a-series-of-tafro-syndrome/. Accessed December 8, 2021.
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