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2020 ISTH TTP Pocket Guideline with GPS

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11 Statement 4 ➤ Due to the severity and instability of their illness, TTP patients experiencing an acute event are often managed in a setting with critical / intensive care capabilities, including continuous monitoring of neurologic status, cardiac status, and oxygen saturation. Initial management in a critical / intensive care setting is considered appropriate on the grounds that TTP patients may deteriorate quickly, and have a high risk of severe organ dysfunction such as coma, stroke, seizures, myocardial infarction, congestive heart failure, arrhythmias, mesenteric ischemia, pancreatitis or acute kidney injury, all of which require early detection, intensive monitoring, and rapid treatment; furthermore, TPE is associated with rare adverse effects that are best detected and managed in the critical / intensive care setting. Statement 5 ➤ We did not systematically search and review the evidence on different modalities of monitoring in TTP patients. In general, the clinical evaluation of TTP patients at the time of hospital admission, and regularly thereafter, emphasizes cardiac and neurological assessment. Specifically, cardiac troponin levels are measured at diagnosis, followed by serial troponin levels, electrocardiography, and echocardiography as clinically indicated. Increased cardiac troponin (>0.25 μg/L) appears to be associated with increased cardiac and cerebral involvement, stroke, and mortality in TTP, although most patients with an increased level of cardiac troponin are asymptomatic. Statement 6 ➤ TTP patients often need a central venous access secured urgently. Rapid placement of central venous access allows TPE to be started as soon as possible. The type of central venous access depends on the modality of TPE: centrifugal apheresis versus membrane filtration. Centrifugal apheresis involves lower blood flow rates, and enables the use of catheters with smaller diameters, and more flexible walls (such as peripheral catheters or standard triple lumen central venous catheters (CVCs). Conversely, membrane filtration involves higher blood flow rates, which requires the use of larger diameter, stiffer catheters (such as standard dialysis catheters or single lumen CVCs). Clinicians should be aware of, or consult the appropriate service for what modality of TPE is used at their center, so appropriate central venous access can be secured before initiation of TPE.

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