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

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.

A case of acquired Thrombotic Thrombocytopenic Purpura (TTP) caused by acute severe hepatitis E: successfully treated by plasma exchange and rituximab

“…To date, we have not seen such a special case of HEV-ALF with TTP in the intensive care unit. In fact, it was already recommended that patients with severe TTP should be admitted to the ICU as soon as possible to get comprehensive treatment 19,20 . In the present case, patient in EICU was closely monitored while receiving full treatment, including ADAMTS13 testing, liver function, kidney function, heart function and blood clotting indicators.…”

Reversible skin microvascular hyporeactivity in patients with immune-mediated thrombocytopenic thrombotic purpura

“…Next, as a control group, we used a previously published cohort of young diabetic patients after correction of ketoacidosis where we showed that microvascular hyporeactivity recovered after acidosis correction [ 14 ] Given that patients with cardiovascular risk factors are susceptible to a lower microvascular reactivity; difference between healthy subjects and iTTP patients could be even more important than differences between diabetic and iTTP patients [ 32 – 35 ]. The device used in this study only allows exploration of the skin microvasculature and we did not investigate the endothelium of key organs affected by iTTP such as the brain, heart and kidney microcirculation [ 36 , 37 ]. Finally, we did not explore the endothelium-independent vasodilation, which requires either heating or nitroprusside challenge.…”