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Early posttransplant plasma ADAMTS13 activity reduction in stem cell transplantation: a prospective study of 46 pediatric patients

Bone Marrow Transplantation volume 54, pages 1926–1929 (2019)Cite this article

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Damage to both endothelial and coagulation systems has been shown to occur after stem cell transplantation (SCT) [1, 2]. A disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) is a von Willebrand factor-cleaving protease that controls the multimer size of von Willebrand factor (VWF). Severe deficiency of this protease can cause thrombotic thrombocytopenic purpura (TTP). There has been accumulating evidence on the decreased ADAMTS13 activity in thrombotic microangiopathies other than TTP [3, 4]. Several reports have suggested that ADAMTS13 can be useful as a clue for the differential diagnosis of microangiopathy [5] and as a potential predictor for developing hepatic sinusoidal obstruction syndrome (SOS) [6]. Little information is available, however, on the posttransplant ADAMTS13 activity changes in patients, especially children, receiving SCT. Our objective was to characterize the time course changes in plasma ADAMTS13 activity during the early posttransplant phase in patients who underwent SCT in a prospective, single-center study.

A total of 46 consecutive patients (age 0–17 years) who underwent SCT at Kobe Children’s Hospital between October 2010 and June 2013 were enrolled. Baseline demographic information and clinical information were obtained from their medical records. Patients with all-cause death within 4 weeks posttransplant were excluded. Informed consent was obtained from the patients or their parents, and the study was approved by the Institutional Review Board of Kobe Children’s Hospital. ADAMTS13 activity, ADAMTS13 inhibitor, antithrombin III, protein C, protein S, tissue plasminogen activator inhibitor-1, and thrombin antithrombin complex in plasma were monitored prospectively before and after SCT. Plasma ADAMTS13 activity was measured with ELISA using a specific murine monoclonal antibody [7]. The ADAMTS13 inhibitor assay was performed using a mixing study. Blood samples were obtained from central venous catheters at three time points (TPs): before (TP 0), 2 weeks after (TP 1), and 4 weeks after (TP 2) transplantation.

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Authors and Affiliations

  1. Department of Hematology and Oncology, Children’s Cancer Center, Kobe Children’s Hospital, Kobe, Japan

    Kenji Kishimoto, Daiichiro Hasegawa, Akihiro Tamura, Nobuyuki Yamamoto, Atsuro Saito, Aiko Kozaki, Toshiaki Ishida & Yoshiyuki Kosaka

  2. Department of Pediatrics, Kita-Harima Medical Center, Ichibacho, Ono, Japan

    Keiichiro Kawasaki

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  1. Kenji Kishimoto
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Correspondence to Kenji Kishimoto.

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Kishimoto, K., Hasegawa, D., Kawasaki, K. et al. Early posttransplant plasma ADAMTS13 activity reduction in stem cell transplantation: a prospective study of 46 pediatric patients. Bone Marrow Transplant 54, 1926–1929 (2019). https://doi.org/10.1038/s41409-019-0506-4

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