Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy

doi:10.1186/s12959-018-0168-2

Review

. 2018 Jul 11:16:14.

doi: 10.1186/s12959-018-0168-2. eCollection 2018.

Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy

Hideo Wada¹, Takeshi Matsumoto², Kei Suzuki³, Hiroshi Imai³, Naoyuki Katayama⁴, Toshiaki Iba⁵, Masanori Matsumoto⁶

Affiliations

¹ 1Department of Molecular and Laboratory Medicine, Mie University Graduate School of Medicine, Tsu, Mie 514-8507 Japan.
² 2Division of Blood Transfusion Medicine and Cell Therapy, Mie University Graduate School of Medicine, Tsu, Japan.
³ 3Emergency Critical Care Center, Mie University Graduate School of Medicine, Tsu, Japan.
⁴ 4Department of Hematology and Oncology, Mie University Graduate School of Medicine, Tsu, Japan.
⁵ 5Department of Emergency and Disaster Medicine, Juntendo University Graduate School of Medicine, Tokyo, Japan.
⁶ 6Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan.

PMID: 30008620
PMCID: PMC6040080
DOI: 10.1186/s12959-018-0168-2

Review

Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy

Hideo Wada et al. Thromb J. 2018.

. 2018 Jul 11:16:14.

doi: 10.1186/s12959-018-0168-2. eCollection 2018.

Authors

Hideo Wada¹, Takeshi Matsumoto², Kei Suzuki³, Hiroshi Imai³, Naoyuki Katayama⁴, Toshiaki Iba⁵, Masanori Matsumoto⁶

Affiliations

¹ 1Department of Molecular and Laboratory Medicine, Mie University Graduate School of Medicine, Tsu, Mie 514-8507 Japan.
² 2Division of Blood Transfusion Medicine and Cell Therapy, Mie University Graduate School of Medicine, Tsu, Japan.
³ 3Emergency Critical Care Center, Mie University Graduate School of Medicine, Tsu, Japan.
⁴ 4Department of Hematology and Oncology, Mie University Graduate School of Medicine, Tsu, Japan.
⁵ 5Department of Emergency and Disaster Medicine, Juntendo University Graduate School of Medicine, Tokyo, Japan.
⁶ 6Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan.

PMID: 30008620
PMCID: PMC6040080
DOI: 10.1186/s12959-018-0168-2

Abstract

Introduction: Both disseminated intravascular coagulation (DIC) and thrombotic microangiopathy (TMA) cause microvascular thrombosis associated with thrombocytopenia, bleeding tendency and organ failure.

Reports and discussion: The frequency of DIC is higher than that of thrombotic thrombocytopenic purpura (TTP). Many patients with TMA are diagnosed with DIC, but only about 15% of DIC patients are diagnosed with TMA. Hyperfibrinolysis is observed in most patients with DIC, and microangiopathic hemolytic anemia is observed in most patients with TMA. Markedly decreased ADAMTS13 activity, the presence of Shiga-toxin-producing Escherichia coli (STEC) and abnormality of the complement system are useful for the diagnosis of TTP, STEC-hemolytic uremic syndrome (HUS)and atypical HUS, respectively. However, there are no specific biomarkers for the diagnosis of DIC.

Conclusion: Although DIC and TMA are similar appearances, all coagulation, fibrinolysis and platelet systems are activated in DIC, and only platelets are markedly activated in TMA.

Keywords: DIC; Hyperfibrinolysis; Microangiopathic hemolytic anemia; Microvascular thrombosis; Organ failure; TMA.

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Conflict of interest statement

The study protocol was approved by the Human Ethics Review Committee of the Mie University School of Medicine, and a signed consent form was obtained from each subject. This study was faithfully carried out in accordance with the principles of the Declaration of Helsinki.A signed consent form was obtained from each subject.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

**Fig. 1**
Concept of DIC and TMA. DIC, disseminated intravascular coagulation; TMA, thrombotic microangiopahy; MHA, microangiopathic hemolyitc anemia; FRMs; fibrin related markers

**Fig. 2**
Mechanism underlying onset for DIC or TMA. DIC, disseminated intravascular coagulation; TMA, thrombotic microangiopahy; TF, tissue factor; ULM-VWF, ultra-large multimers of von Willebrand factor

**Fig. 3**
Microvascular thrombosis in DIC and TMA. DIC, disseminated intravascular coagulation; TMA, thrombotic microangiopahy

See this image and copyright information in PMC

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References

1. Taylor FB, Jr, Toh CH, Hoots WK, Wada H, Levi M. Scientific subcommittee on disseminated intravascular coagulation (DIC) of the international society on thrombosis and Haemostasis (ISTH): towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost. 2001;86:1327–1330. doi: 10.1055/s-0037-1616068. - DOI - PubMed
1. Wada H, Matsumoto T, Yamashita Y, Hatada T. Disseminated intravascular coagulation: testing and diagnosis. Clin Chim Acta. 2014;436C:130–134. doi: 10.1016/j.cca.2014.04.020. - DOI - PubMed
1. Wada H, Matsumoto T, Yamashita Y. Natural history of thrombotic thrombocytopenic Purpura and hemolytic uremic syndrome. Semin Thromb Hemost. 2014;40:866–873. doi: 10.1055/s-0034-1395154. - DOI - PubMed
1. Matsumoto M, Fujimura Y, Wada H, Kokame K, Miyakawa Y, Ueda Y, Higasa S, Moriki T, Yagi H, Miyata T, Murata M. For TTP group of blood coagulation abnormalities research team, research on rare and intractable disease supported by health, labour, and welfare sciences research grants.: Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. Int J Hematol. 2017;106:3–15. doi: 10.1007/s12185-017-2264-7. - DOI - PubMed
1. South K, Lane DA. ADAMTS-13 and von Willebrand factor: a dynamic duo. J Thromb Haemost. 2017; in press - PMC - PubMed

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[1] Taylor FB, Jr, Toh CH, Hoots WK, Wada H, Levi M. Scientific subcommittee on disseminated intravascular coagulation (DIC) of the international society on thrombosis and Haemostasis (ISTH): towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost. 2001;86:1327–1330. doi: 10.1055/s-0037-1616068. - DOI - PubMed

[2] Taylor FB, Jr, Toh CH, Hoots WK, Wada H, Levi M. Scientific subcommittee on disseminated intravascular coagulation (DIC) of the international society on thrombosis and Haemostasis (ISTH): towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost. 2001;86:1327–1330. doi: 10.1055/s-0037-1616068. - DOI - PubMed

[3] Wada H, Matsumoto T, Yamashita Y, Hatada T. Disseminated intravascular coagulation: testing and diagnosis. Clin Chim Acta. 2014;436C:130–134. doi: 10.1016/j.cca.2014.04.020. - DOI - PubMed

[4] Wada H, Matsumoto T, Yamashita Y, Hatada T. Disseminated intravascular coagulation: testing and diagnosis. Clin Chim Acta. 2014;436C:130–134. doi: 10.1016/j.cca.2014.04.020. - DOI - PubMed

[5] Wada H, Matsumoto T, Yamashita Y. Natural history of thrombotic thrombocytopenic Purpura and hemolytic uremic syndrome. Semin Thromb Hemost. 2014;40:866–873. doi: 10.1055/s-0034-1395154. - DOI - PubMed

[6] Wada H, Matsumoto T, Yamashita Y. Natural history of thrombotic thrombocytopenic Purpura and hemolytic uremic syndrome. Semin Thromb Hemost. 2014;40:866–873. doi: 10.1055/s-0034-1395154. - DOI - PubMed

[7] Matsumoto M, Fujimura Y, Wada H, Kokame K, Miyakawa Y, Ueda Y, Higasa S, Moriki T, Yagi H, Miyata T, Murata M. For TTP group of blood coagulation abnormalities research team, research on rare and intractable disease supported by health, labour, and welfare sciences research grants.: Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. Int J Hematol. 2017;106:3–15. doi: 10.1007/s12185-017-2264-7. - DOI - PubMed

[8] Matsumoto M, Fujimura Y, Wada H, Kokame K, Miyakawa Y, Ueda Y, Higasa S, Moriki T, Yagi H, Miyata T, Murata M. For TTP group of blood coagulation abnormalities research team, research on rare and intractable disease supported by health, labour, and welfare sciences research grants.: Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. Int J Hematol. 2017;106:3–15. doi: 10.1007/s12185-017-2264-7. - DOI - PubMed

[9] South K, Lane DA. ADAMTS-13 and von Willebrand factor: a dynamic duo. J Thromb Haemost. 2017; in press - PMC - PubMed

[10] South K, Lane DA. ADAMTS-13 and von Willebrand factor: a dynamic duo. J Thromb Haemost. 2017; in press - PMC - PubMed

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Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy

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Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy

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