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Thrombotic microangiopathy caused by severe graft dysfunction after living donor liver transplantation: report of a case

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Abstract

Thrombotic microangiopathy (TMA) is a life-threatening complication after transplantation including liver transplantation, and its typical clinical picture is characterized by hemolytic anemia, thrombocytopenia, renal dysfunction, neurological abnormalities, and fever. We report the case of a 56-year-old female with end-stage liver disease who underwent living donor liver transplantation (LDLT), and whose postoperative course was characterized by renal failure and progressive hyperbilirubinemia. Two weeks after LDLT, she started to show progressive thrombocytopenia, anemia, oliguria, and encephalopathy. From these clinical manifestations, she was diagnosed as having TMA and underwent plasma exchanges with continuous hemodialysis under temporary holding calcineurin inhibitors. The patient promptly responded to the treatment, with improved hematological, hepatic, and renal conditions, and was discharged from hospital a month later in a stable condition. We describe this case of TMA after LDLT with poor graft function and extensively review the disease in liver transplant recipients.

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Abbreviations

TMA:

Thrombotic microangiopathy

LDLT:

Living donor liver transplantation

AIH:

Autoimmune hepatitis

PBC:

Primary biliary cirrhosis

CNI:

Calcineurin inhibitor

POD:

Postoperative day

PE:

Plasma exchange

CHDF:

Continuous hemodiafiltration

ADAMTS13:

A disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13

vWF:

von Willebrand factor

References

  1. Ikegami T, Shimada M, Imura S, Arakawa Y, Nii A, Morine Y, et al. Current concept of small-for-size grafts in living donor liver transplantation. Surg Today. 2008;38:971–82.

    Article  PubMed  Google Scholar 

  2. Lee SG, Hwang S, Kim KH, Ahn CS, Moon DB, Ha TY, et al. Toward 300 liver transplants a year. Surg Today. 2009;39:367–73.

    Article  CAS  PubMed  Google Scholar 

  3. Moake JL. Thrombotic microangiopathies. N Engl J Med. 2002;22(347):589–600.

    Article  Google Scholar 

  4. Ferrari S, Scheiflinger F, Rieger M, Mudde G, Wolf M, Coppo P, et al. Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity. Blood. 2007;109:2815–22.

    CAS  PubMed  Google Scholar 

  5. Tamura S, Sugawara Y, Matsui Y, Kishi Y, Akamatsu N, Kaneko J, et al. Thrombotic microangiopathy in living-donor liver transplantation. Transplantation. 2005;80:169–75.

    Article  PubMed  Google Scholar 

  6. Crawley JT, de Groot R, Xiang Y, Luken BM, Lane DA. Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor. Blood. 2011;118:3212–21.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  7. Kamioka I, Nozu K, Fujita T, Kaito H, Tanaka R, Yoshiya K, et al. Prognosis and pathological characteristics of five children with non-Shiga toxin-mediated hemolytic uremic syndrome. Pediatr Int. 2007;49:196–201.

    Article  PubMed  Google Scholar 

  8. Trimarchi HM, Truong LD, Brennan S, Gonzalez JM, Suki WN. FK506-associated thrombotic microangiopathy: report of two cases and review of the literature. Transplantation. 1999;67:539–44.

    Article  CAS  PubMed  Google Scholar 

  9. Ponticelli C, Banfi G. Thrombotic microangiopathy after kidney transplantation. Transpl Int. 2006;19:789–94.

    Article  PubMed  Google Scholar 

  10. Ikegami T, Imai D, Wang H, Yoshizumi T, Yamashita YI, Ninomiya M, et al. D-MELD as a predictor of early graft mortality in adult-to-adult living-donor liver transplantation. Transplantation. 2013 [Epub ahead of print].

  11. Ikegami T, Shirabe K, Yoshizumi T, Aishima S, Taketomi YA, Soejima Y, et al. Primary graft dysfunction after living donor liver transplantation is characterized by delayed functional hyperbilirubinemia. Am J Transplant. 2012;12:1886.

    Article  CAS  PubMed  Google Scholar 

  12. Nakazawa Y, Hashikura Y, Urata K, Ikegami T, Terada M, Yagi H, et al. Von Willebrand factor-cleaving protease activity in thrombotic microangiopathy after living donor liver transplantation: a case report. Liver Transpl. 2003;9:1328–33.

    Article  PubMed  Google Scholar 

  13. Uemura M, Tatsumi K, Matsumoto M, Fujimoto M, Matsuyama T, Ishikawa M, et al. Localization of ADAMTS13 to the stellate cells of human liver. Blood. 2005;106:922–4.

    Article  CAS  PubMed  Google Scholar 

  14. Kobayashi T, Wada H, Usui M, Sakurai H, Matsumoto T, Nobori T, et al. Decreased ADAMTS13 levels in patients after living donor liver transplantation. Thromb Res. 2009;124:541.

    Article  CAS  PubMed  Google Scholar 

  15. Kuriyama N, Isaji S, Hamada T, Kishiwada M, Ohsawa I, Usui M, et al. The cryoprotective effects of addition of activated protein C into preservation solution on small-for-size grafts in rats. Liver Transpl. 2010;16:1–11.

    Article  PubMed  Google Scholar 

  16. Shindoh J, Sugawara Y, Akamatsu N, Kaneko J, Tamura S, Yamashiki N, et al. Thrombotic microangiopathy after living-donor liver transplantation. Am J Transplant. 2012;12:728–36.

    Article  CAS  PubMed  Google Scholar 

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Disclosures

Conflict of Interest:

The authors declare that they have no conflict of interest.

Human/Animal Rights:

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008(5).

Informed Consent:

Informed consent was obtained from all patients for being included in the study.

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Correspondence to Takeo Toshima.

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Matsuda, D., Toshima, T., Ikegami, T. et al. Thrombotic microangiopathy caused by severe graft dysfunction after living donor liver transplantation: report of a case. Clin J Gastroenterol 7, 159–163 (2014). https://doi.org/10.1007/s12328-013-0446-2

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  • DOI: https://doi.org/10.1007/s12328-013-0446-2

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