TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease


  • Gordan Srkalovic Sparrow Cancer Center, Edward W. Sparrow Hospital Association, Lansing, MI, USA
  • Inga Marijanovic Oncology Clinic, University Clinical Hospital Mostar, Mostar, Bosnia and Herzegovina
  • Maya B Srkalovic Department of Biology, Kalamazoo College, Kalamazoo, MI, USA
  • David C Fajgenbaum Divison of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA




TAFRO, Castleman disease, lymphoproliferative disorders, interleukin-6, cytokines, cytokine storm


Castleman disease (CD) describes a group of three rare and poorly understood lymphoproliferative disorders that have heterogeneous clinical symptoms and common lymph node histopathological features. Unicentric CD (UCD) involves a single region of enlarged nodes. Multicentric CD (MCD) involves multiple regions of enlarged lymph nodes, constitutional symptoms, and organ dysfunction due to a cytokine storm often including interleukin 6. MCD is further divided into Human Herpes Virus-8 (HHV-8)-associated MCD, which occurs in immunocompromised individuals, and HHV-8-negative/idiopathic MCD (iMCD). Recently, iMCD has been further sub-divided into patients with TAFRO syndrome, which involves thrombocytopenia (T), anasarca (A), fevers (F), reticulin myelofibrosis (R), organomegaly (O), and normal or only slightly elevated immunoglobulin levels, and those who do not have TAFRO syndrome. Non-TAFRO iMCD patients typically have thrombocytosis, less severe fluid accumulation, and hypergammaglobulinemia. iMCD patients with TAFRO syndrome may have a worse prognosis, but more research is needed.


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Author Biography

  • Maya B Srkalovic, Department of Biology, Kalamazoo College, Kalamazoo, MI, USA
    Department of Biology


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TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease


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TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease. Biomol Biomed [Internet]. 2017 May 20 [cited 2024 May 19];17(2):81-4. Available from: https://www.bjbms.org/ojs/index.php/bjbms/article/view/1930