Advertisement

CEN Case Reports

, Volume 7, Issue 2, pp 243–247 | Cite as

Thrombotic microangiopathy on kidney biopsy in a patient with TAFRO syndrome

  • Toshikazu Ozeki
  • Masashi Tsuji
  • Junichiro Yamamoto
  • Chisato Shigematsu
  • Shoichi Maruyama
Case Report

Abstract

TAFRO syndrome represents a characteristic constellation of symptoms comprising Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, and Organomegaly, and is considered to be a clinicopathologic variant of idiopathic multicentric Castleman disease. A 51-year-old woman was admitted to the hospital complaining of abdominal distension. Findings on physical examination were indicative of anasarca. Computed tomography revealed mild splenomegaly, pericardial effusion, pleural effusion, ascites, and paraaortic lymphadenopathy. Blood tests showed thrombocytopenia, and urinalysis demonstrated hematuria, proteinuria, and worsening renal function. Kidney biopsy was performed and revealed thrombotic microangiopathy-like lesions with global sclerosis of 1 of the 16 glomeruli on light microscopy. The remaining glomeruli had a distinct lobular pattern, with mesangiolysis, double contours of the glomerular basement membranes, and marked endothelial swelling. Immunofluorescence studies for IgG, IgM, IgA, C1q, C3, C4, κ-light chains, and λ-light chains were indeterminate. Electron microscopy showed marked endothelial swelling. We made a diagnosis of TAFRO syndrome and started steroid treatment, following which her symptoms gradually improved. There are few reports describing renal pathology in a patient with TAFRO syndrome.

Keywords

TAFRO syndrome Renal pathology Thrombotic microangiopathy 

Notes

Funding

None.

Compliance with ethical standards

Conflict of interest

All the authors have declared no competing interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

Informed consent was obtained from the patient in the case report.

References

  1. 1.
    Takai K, Nikkuni K, Shibuya H, Hashidate H. Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly. Rinsho Ketsueki. 2010;51:320–5 (in Japanese).PubMedGoogle Scholar
  2. 2.
    Masaki Y, Nakajima A, Iwao H, Kurose N, Sato T, Nakamura T, et al. Japanese variant of multicentric Castleman’s disease associated with serositis and thrombocytopenia—a report of two cases: is TAFRO syndrome (Castleman-Kojima disease) a distinct clinicopathological entity? J Clin Exp Hematop. 2013;53:79–85.CrossRefPubMedGoogle Scholar
  3. 3.
    Inoue M, Ankou M, Hua J, Iwaki Y, Hagihara M. Complete resolution of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) after immunosuppressive therapies using corticosteroids and cyclosporin A: a case report. J Clin Exp Hematop. 2013;53:95–9.CrossRefPubMedGoogle Scholar
  4. 4.
    Kubokawa I, Yachie A, Hayakawa A, Hirase S, Yamamoto N, Mori T, et al. The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multi centric Castleman’s disease. BMC Pediatr. 2014;14:139.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Fujiki T, Hirasawa S, Watanabe S, Iwamoto S, Ando R. Successful treatment by tocilizumab without steroid in a very severe case of TAFRO syndrome. CEN Case Rep. 2017;6:105–10.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Masaki Y, Kawabata H, Takai K, et al. Proposed diagnostic criteria, disease severity classification and treatment strategy of TAFRO syndrome, 2015 version. Int J Hematol. 2016;103:686–92.CrossRefPubMedGoogle Scholar
  7. 7.
    El Karoui K, Vuiblet V, Dion D, Izzedine H, Guitard J, et al. Renal involvement in Castleman disease. Nephrol Dial Transpl. 2011;26(2):599–609.CrossRefGoogle Scholar
  8. 8.
    Xu D, Lv J, Dong Y, Wang S, Su T, et al. Renal involvement in a large cohort of Chinese patients with Castleman disease. Nephrol Dial Transpl. 2012;27:119–25.CrossRefGoogle Scholar
  9. 9.
    José FF, Kerbauy LN, Perini GF, et al. A life-threatening case of TAFRO syndrome with dramatic response to tocilizumab, rituximab, and pulse steroids. Medicine (Baltimore). 2017;96(13):e6271.CrossRefGoogle Scholar
  10. 10.
    Eremina V, Jefferson JA, Kowalewska J, et al. VEGF inhibition and renal thrombotic microangiopathy. N Engl J Med. 2008;358:1129–36.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Sartelet H, Toupance O, Lorenzato M, et al. Sirolimus-induced thrombotic microangiopathy is associated with decreased expression of vascular endothelial growth factor in kidneys. Am J Transpl. 2005;5:2441–7.CrossRefGoogle Scholar
  12. 12.
    Mutneja A, Cossey LN, Liapis H, Chen YM. A rare case of renal thrombotic microangiopathy associated with Castleman’s disease. BMC Nephrol. 2017;18(1):57.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  1. 1.Department of NephrologyTsushima City HospitalTsushimaJapan
  2. 2.Department of NephrologyNagoya University Graduate School of MedicineNagoyaJapan

Personalised recommendations