TAFRO syndrome: A case report and review of the literature

• Type of Material: Article
• Authors: Tieying Hou, SUNY Buffalo; Jaspreet Dhillon, Erie County Medical Center; Wenbin Xiao, National Cancer Institute; Elaine S. Jaffe, National Cancer Institute; Amy M. Sands, SUNY Buffalo; Vishala Neppalli, Roswell Park Cancer Institute; George Deeb, Emory University; Nan Zhang, SUNY Buffalo
• Language: English
• Date: 2017-11-01
• Publisher: Elsevier
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2017 The Authors
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.ehpc.2017.04.003
• Title of Journal or Parent Work: Human Pathology: Case Reports
• Volume: 10
• Start Page: 1
• End Page: 4
• Keywords: ascites; inerleukin-6; TAFRO syndrome; Castleman disease; thrombocytopenia; anemia; myelofibrosis; fever; organomegaly; renal dysfunction; variant
• Research Categories: Biology, Virology; Health Sciences, Epidemiology

Abstract

TAFRO syndrome is a rare clinicopathologic variant of idiopathic multicentric Castleman disease characterized by Thrombocytopenia, Ascites (anasarca), myeloFibrosis, Renal dysfunction, and Organomegaly. Here, we report a case of TAFRO syndrome in an HIV-negative young Caucasian male who presented with fever, normocytic anemia, thrombocytopenia, and acute renal insufficiency. The serum interleukin-6 (IL-6) level was elevated. Chest and abdominal CT revealed bilateral pleural effusion, ascites, splenomegaly, and multiple mildly enlarged lymph nodes. An excisional biopsy of inguinal lymph node showed a few atrophic follicles and expansion of interfollicular areas by marked vascular proliferation and polytypic plasmacytosis. HHV-8 was negative. Subsequent bone marrow biopsy was normocellular with moderately increased megakaryocytes and occasional megakaryocytic emperipolesis. His signs and symptoms improved after treatment with methylprednisolone and tocilizumab (anti-IL-6 receptor antibody). Our study confirms the distinctive nature of this syndrome, which should allow for better recognition and appropriate therapy.