CEN Case Reports

, Volume 8, Issue 1, pp 1–7 | Cite as

Thrombotic microangiopathy associated with anticardiolipin antibody in a kidney transplant recipient with polycythemia

  • Akihiro Tsuchimoto
  • Yuta Matsukuma
  • Kenji Ueki
  • Takehiro Nishiki
  • Atsushi Doi
  • Yasuhiro Okabe
  • Masafumi Nakamura
  • Kazuhiko Tsuruya
  • Toshiaki NakanoEmail author
  • Takanari Kitazono
  • Kosuke Masutani
Case Report


Thrombotic microangiopathy (TMA) develops from various etiologies, and it is often difficult to distinguish the etiology of TMA in kidney transplantation. Antiphospholipid syndrome (APS) is one of the differential diagnoses for TMA that may cause acute loss of graft function or fatal thrombotic complications. This report details a 66-year-old male patient with polycythemia after ABO-incompatible kidney transplantation. Antibody screening tests were negative before transplant. Despite administration of an adequate desensitization therapy including plasmapheresis and rituximab, he developed acute graft dysfunction on postoperative day 112 and graft biopsy revealed prominent microvascular inflammation in the glomerular capillaries without immunoglobulin deposits. Flow cytometric panel-reactive antibody screening failed to detect donor-specific antibodies at both pre-transplant and episode biopsies. Anticardiolipin antibody was repeatedly positive, but neither thrombosis nor previous thrombotic episodes were detected. After excluding several differential diagnoses, the graft dysfunction with unexplained TMA was treated with steroid pulse, plasmapheresis and rituximab re-induction. Anticardiolipin antibody disappeared after this intensive treatment and graft function recovered gradually and stabilized for 52 months. This report suggests that asymptomatic anticardiolipin antibody may be associated with acute graft dysfunction. Even if thrombotic episodes are not observed, an exist of anticardiolipin antibody may be one of the risk factors of renal TMA after kidney transplantation.


Living-donor Antiphospholipid syndrome Myeloproliferative disorders Rituximab Plasma exchange Plasmapheresis Graft biopsy 



Antiphospholipid syndrome


β2glycoprotein I


Thrombotic microangiopathy



We thank Gillian Campbell, PhD, from Edanz Group ( for editing a draft of this manuscript.

Author contributions

TA participated in planning this research and data collection, MK, MY and UK analyzed the renal pathology, NT and DA participated in data collection, and YO, NM, TK, NT, and KT participated in the management of this research. All authors contributed to the writing of the manuscript.


Honoraria Takanari Kitazono (Bayer Pharmaceutical Co., Bristol-Myers Squibb Co., Daiichi-Sankyo Co.), Kazuhiko Tsuruya (Chugai Pharmaceutical Co., Kyowa Hakko Kirin Co.). Donations Takanari Kitazono (Astellas Pharma Inc., Daiichi-Sankyo Co., Eisai Co., Kyowa Hakko Kirin Co., Mitsubishi Tanabe Pharma Co., MSD K.K., Ono Pharmaceutical Co., Otsuka Pharmaceutical Co., Sanofi-Aventis Pharmaceutical Co., Takeda Pharmaceutical Co.), Kazuhiko Tsuruya (Chugai Pharmaceutical Co., Kyowa Hakko Kirin Co., Otsuka Pharmaceutical Co., Takeda Pharmaceutical Co.). Endowed department Kazuhiko Tsuruya (Baxter).

Compliance with ethical standards


The clinical and research activities of the study were consistent with the principles of the Declaration of Istanbul, as outlined in the Declaration of Istanbul on Organ Trafficking and Transplant Tourism. Informed consent was acquired from the participant included in the study.

Conflict of interest

The authors have no conflict of interest to declare.

Research involving human participants

Ethical Approval: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee at which the studies were conducted (IRB approval number #24–54) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  • Akihiro Tsuchimoto
    • 1
  • Yuta Matsukuma
    • 1
  • Kenji Ueki
    • 1
  • Takehiro Nishiki
    • 2
  • Atsushi Doi
    • 3
  • Yasuhiro Okabe
    • 3
  • Masafumi Nakamura
    • 3
  • Kazuhiko Tsuruya
    • 1
  • Toshiaki Nakano
    • 1
    • 4
    Email author
  • Takanari Kitazono
    • 1
  • Kosuke Masutani
    • 5
  1. 1.Department of Medicine and Clinical Science, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  2. 2.Department of SurgeryFukuoka Red Cross HospitalFukuokaJapan
  3. 3.Department of Surgery and OncologyKyushu UniversityFukuokaJapan
  4. 4.Department of Integrated Therapy for Chronic Kidney DiseaseKyushu UniversityFukuokaJapan
  5. 5.Division of Nephrology and Rheumatology, Department of Internal Medicine, Faculty of MedicineFukuoka UniversityFukuokaJapan

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