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Strong association between insufficient plasma exchange and fatal outcomes in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura

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Abstract

Plasma exchange (PEX) using fresh frozen plasma has considerably reduced the mortality rate in patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP). However, some patients still do not survive even with treatment, but little information is available regarding which treatment these patients received. This study was conducted to obtain this information in 240 patients who met the current iTTP diagnostic criteria and completed at least 30 days of follow-up except for deceased cases. These patients were divided into three groups: survivors (n = 195), TTP-related deaths (n = 32), and other cause of death (n = 13). In the TTP-related death group, 26 of 32 patients experienced sudden death, mostly following radical hypotension and bradycardia. The median follow-up time after admission was 5.0 days, and the median number of PEX sessions was 2.5. Nine patients underwent autopsy and had cardiac microvascular thrombi in arterioles. Levels of lactate dehydrogenase, total bilirubin, serum creatinine, and d-dimer were significantly higher in the TTP-related death group than in the survivors group. Frequent PEX (> 20 sessions) was not associated with TTP-related death. In the acute phase of iTTP, patients with substantial organ damage caused by microthrombi have a greater mortality risk, even after just a few PEX sessions.

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Acknowledgements

The authors would like to thank all physicians for sending the data and samples of the patients with iTTP.

Funding

This was supported by research grants from the Ministry of Health, Labour, and Welfare of Japan.

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Author notes

  1. Michinori Kayashima and Kazuya Sakai contributed equally to this work.

Authors and Affiliations

  1. Department of Blood Transfusion Medicine, Nara Medical University, 840 Shijo-Cho, Kashihara, 634-8522, Japan

    Michinori Kayashima, Kazuya Sakai, Masayuki Kubo, Eriko Hamada, Masaki Hayakawa & Masanori Matsumoto

  2. Department of Forensic Medicine, National Defense Medical College, Tokorozawa, Japan

    Kazuki Harada & Jun Kanetake

  3. Department of Pathology, National Cerebral and Cardiovascular Center, Suita, Japan

    Kinta Hatakeyama

Authors
  1. Michinori Kayashima
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  2. Kazuya Sakai
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  3. Kazuki Harada
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  4. Jun Kanetake
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  5. Masayuki Kubo
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  6. Eriko Hamada
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  7. Masaki Hayakawa
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  8. Kinta Hatakeyama
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  9. Masanori Matsumoto
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Contributions

MK treated the patients, collected patient data, and wrote the manuscript; KS designed the study concept, analyzed data, and wrote the manuscript; IH, JK, and KH performed the histological analysis; MK, EH, and MH collected patient data; MM directed the study and wrote the manuscript.

Corresponding author

Correspondence to Masanori Matsumoto.

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Conflict of interest

M. Matsumoto is a member of the clinical advisory board of Takeda Yakuhin and Sanofi. He is also an inventor of the ADAMTS13 act-ELISA. The remaining authors declare no competing financial interests.

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Kayashima, M., Sakai, K., Harada, K. et al. Strong association between insufficient plasma exchange and fatal outcomes in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura. Int J Hematol 114, 415–423 (2021). https://doi.org/10.1007/s12185-021-03197-5

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  • DOI: https://doi.org/10.1007/s12185-021-03197-5

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