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Review
. 2022 Feb 19;11(4):1103.
doi: 10.3390/jcm11041103.

Severe Thrombotic Thrombocytopenic Purpura (TTP) with Organ Failure in Critically Ill Patients

Sofiane Fodil  1 , Lara Zafrani  1
Affiliations
Review

Severe Thrombotic Thrombocytopenic Purpura (TTP) with Organ Failure in Critically Ill Patients

Sofiane Fodil et al. J Clin Med. .

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a multiorgan disorder. Organ dysfunction occurs as a consequence of widespread microvascular thrombosis, especially in the heart, brain and kidney, causing transient or partial occlusion of vessels, resulting in organ ischemia. Intensive care unit (ICU) admission varies between 40% and 100% of patients with TTP, either because of severe organ failure or in order to initiate emergency plasma exchange (PEx). Severe neurologic manifestations and cardiac involvement have been associated with higher mortality. Acute kidney injury, although usually less severe than that in hemolytic and uremic syndrome, is common during TTP. Initial management in the ICU should always be considered in TTP patients. The current treatment of TTP in the acute phase is based on urgent PEx, combined with corticosteroid therapy, B-cell-targeted immunotherapy, rituximab and inhibition of the interaction between ultra-large Von Willebrand factor multimers and platelets, using caplacizumab, a monoclonal antibody. ICU management permits close monitoring and the rapid introduction of life-sustaining therapies. This review details the epidemiology of TTP in the ICU, organ failures of critically ill patients with TTP, and the initial management of TTP patients in the ICU.

Keywords: intensive care unit; organ failure; thrombotic thrombocytopenic purpura.

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Conflict of interest statement

L.Z. has received a research grant from Jazz Pharmaceuticals, which is not related to this review.

Figures

Figure 1
Figure 1
Organ injury in thrombotic thrombocytopenic purpura (TTP). The main organ involvement in TTP is in the central nervous system (CNS), heart, kidneys, pancreas and macroscopic thrombosis.
Figure 2
Figure 2
Initial management of immune thrombotic thrombocytopenic purpura (TTP). Current treatment of TTP in the acute phase is based on urgent plasma exchange (PEx), combined with corticosteroid therapy, B-cell-targeted immunotherapy, rituximab and inhibition of the interaction between ultralarge VWF multimers and platelets, using caplacizumab, a monoclonal antibody.
See this image and copyright information in PMC

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