International Journal of Hematology

, Volume 103, Issue 6, pp 718–723 | Cite as

Successful treatment of TAFRO syndrome, a variant type of multicentric Castleman disease with thrombotic microangiopathy, with anti-IL-6 receptor antibody and steroids

  • Shiho Fujiwara
  • Hiromi Mochinaga
  • Hirotomo Nakata
  • Koichi Ohshima
  • Masanori Matsumoto
  • Mitsuhiro Uchiba
  • Yoshiki Mikami
  • Hiroyuki Hata
  • Yutaka Okuno
  • Hiroaki Mitsuya
  • Kisato NosakaEmail author
Case Report


TAFRO syndrome is a rare variant type of multicentric Castleman disease, which is characterized by thrombocytopenia, anasarca, reticulin fibrosis of bone marrow, renal dysfunction and organomegaly. Here, we report a case of TAFRO syndrome that was successfully treated with tocilizumab. A 50-year-old man, who presented with fever, epigastric pain, abdominal fullness, and massive edema of the extremities, was admitted to our hospital. Computed tomography revealed bilateral pleural effusions, ascites, and lymphadenopathy. Laboratory data showed renal dysfunction, anemia, and thrombocytopenia. Examination of bone marrow and cervical lymph nodes led to a diagnosis of hyaline vascular-type Castleman disease. The level of serum interleukin (IL)-6 was extremely high. TAFRO syndrome was finally diagnosed. The patient was treated weekly with tocilizumab, an anti-IL-6 receptor antibody and steroids. In 4 weeks, all symptoms disappeared and serum IL-6 level returned to normal. Activity of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), which was significantly decreased (9.9 %) prior to treatment, increased after treatment with tocilizumab. The present case suggests that tocilizumab is an effective therapeutic agent for TAFRO syndrome. We suggest that hypercytokinemia in TAFRO syndrome inhibits ADAMTS13 activity, thereby inducing thrombotic microangiopathy.


TAFRO syndrome Castleman disease Tocilizumab ADAMTS13 


Compliance with ethical standards

Conflict of interest

K. N. has received honoraria for lecture from Chugai Pharmaceutical.


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Copyright information

© The Japanese Society of Hematology 2016

Authors and Affiliations

  • Shiho Fujiwara
    • 1
  • Hiromi Mochinaga
    • 1
  • Hirotomo Nakata
    • 1
  • Koichi Ohshima
    • 2
  • Masanori Matsumoto
    • 3
  • Mitsuhiro Uchiba
    • 4
  • Yoshiki Mikami
    • 5
  • Hiroyuki Hata
    • 6
  • Yutaka Okuno
    • 1
  • Hiroaki Mitsuya
    • 1
  • Kisato Nosaka
    • 1
    • 7
    Email author
  1. 1.Department of HematologyKumamoto University School of MedicineKumamotoJapan
  2. 2.Department of PathologyKurume University School of MedicineFukuokaJapan
  3. 3.Department of Blood Transfusion MedicineNara Medical UniversityNaraJapan
  4. 4.Department of Blood Transfusion and Cell TherapyKumamoto University HospitalKumamotoJapan
  5. 5.Department of Surgical PathologyKumamoto University HospitalKumamotoJapan
  6. 6.Division of Informative Clinical Sciences, Faculty of Medical SciencesKumamoto University School of MedicineKumamotoJapan
  7. 7.Cancer Center of Kumamoto Medical School of MedicineKumamotoJapan

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