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38 - Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome and Disseminated Intravascular Coagulation

from Section 8 - Hematology–Oncology Emergencies

Published online by Cambridge University Press:  02 November 2023

By
Jai Madhok
Edited by
Kaushal Shah  and
Jarone Lee
Edited in association with
Clark G. Owyang  and
Benjamin Christian Renne
Kaushal Shah
Affiliation:
Weill Cornell Medical Center, New York
Jarone Lee
Affiliation:
Massachusetts General Hospital, Boston
Clark G. Owyang
Affiliation:
Weill Cornell Medical Center, New York
Benjamin Christian Renne
Affiliation:
Massachusetts General Hospital, Boston
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Summary

  • Thrombotic microangiopathies (TMA) are a group of conditions characterized by excessive platelet activation, resulting in microvascular thrombi and platelet consumption (thrombocytopenia). The thrombosed microvasculature shears red blood cells (RBCs), causing microangiopathic hemolytic anemia. Primary TMAs include thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), while secondary TMAs result from infection, pregnancy, malignancy, drugs and toxins, among other causes.

  • TMAs do not directly involve the coagulation cascade, and therefore do not prolong coagulation studies. Microthrombi may result in ischemic complications in any organ system.

Keywords

coagulation cascadehemorrhagepetechiaepurpuraplatelets
Type
Chapter
Information
Practical Emergency Resuscitation and Critical Care , pp. 387 - 396
Publisher: Cambridge University Press
Print publication year: 2023

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References

Levi, M. Disseminated intravascular coagulation. Crit Care Med 2007;35:2191–2195.CrossRefGoogle ScholarPubMed
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Levi, M, Toh, CH, Thachil, J, et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. Br J Haematol 2009;145:24–33.CrossRefGoogle ScholarPubMed
Masias, C, Cataland, SR. The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis. Blood 2018;132(9):903–910.CrossRefGoogle ScholarPubMed
Noris, M, Remuzzi, G. Atypical hemolytic-uremic syndrome. N Engl J Med 2009;361(17):1676–1687.CrossRefGoogle ScholarPubMed
Scott, SB. Emergency department management of hematologic and oncologic complications in the patient infected with HIV. Emerg Med Clin North Am 2010;28:325–333.CrossRefGoogle ScholarPubMed

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