• Yumeng Qiao, Xin Zhang, Rong Xu, Xiaoyu Jia, and Qian Wang.
• Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China.
• Medicine (Baltimore). 2024 Aug 2; 103 (31): e39148e39148.

RationaleTAFRO syndrome is a systemic inflammatory disorder, manifesting as thrombocytopenia (t), anasarca (a), fever (f), reticulin myelofibrosis/renal insufficiency (r), and organomegaly (o), and considered as a unique clinical subtype of idiopathic multicentric Castleman disease (iMCD). Such syndrome gave rise to a clinical picture similar to that of either a connective tissue disease or an autoimmune disease.Patient ConcernsA Chinese young female initially presenting with arthralgia, Raynaud phenomenon, generalized edema, and a positive anti-small nuclear ribonucleoprotein particle antibody was diagnosed as mixed connective tissue disease. The kidney biopsy showed thrombotic microangiopathy. Bone marrow smear showed bone marrow hyperplasia and biopsy revealed suspected light chain restricted expression, megakaryocyte proliferation, and moderate to severe bone marrow fibrosis. A lymph node biopsy was conducted and the histopathological findings were consistent with the subtype of mixed Castleman disease. The clinical symptoms were relieved after regular chemotherapy.DiagnosesAfter above examination results and clinical manifestations, the final diagnoses was TAFRO syndrome.InterventionThe she was started on chemotherapy with bortezomib, cyclophosphamide, and dexamethasone.OutcomeAfter chemotherapy, symptoms such as thrombocytopenia, hematuria and proteinuria disappeared, lymphadenopathy and VEGF level decreased, and bone marrow fibrosis relieved.LessonsOur case illustrated the first cases of shared characteristics of mixed connective tissue disease and iMCD-TAFRO syndrome. Cytokines may play a role in the shared pathogenicity of the iMCD-TAFRO syndrome and systemic autoimmune diseases. Therapy directly against inflammatory factors such as corticosteroids or chemotherapy have an important therapeutic implication.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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