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Author Notes:

Corresponding author at: Department of Pathology, Buffalo General Medical Center, SUNY at Buffalo, 100 High St, A749, Buffalo, NY 14206, United States., nzhang@kaleidahealth.org (N. Zhang).

The authors have no conflict of interest to report.

Subjects:

Keywords:

  • TAFRO syndrome
  • variant
  • Castleman disease
  • thrombocytopenia
  • ascites
  • myelofibrosis
  • renal dysfunction
  • organomegaly
  • fever
  • anemia
  • inerleukin-6

TAFRO syndrome: A case report and review of the literature

Tools:

Journal Title:

Human Pathology: Case Reports

Volume:

Volume 10

Publisher:

, Pages 1-4

Type of Work:

Article | Post-print: After Peer Review

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.

Copyright information:

© 2017 The Authors

This is an Open Access work distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (https://creativecommons.org/licenses/by-nc-nd/4.0/).
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