2020
DOI: 10.1111/jth.15010
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Abstract: Background: Despite advances in treatment options for thrombotic thrombocytopenic purpura (TTP), there are still limited high quality data to inform clinicians regarding its appropriate treatment. Methods: In June 2018, the ISTH formed a multidisciplinary guideline panel to issue recommendations about treatment of TTP. The panel discussed 12 treatment questions related to immune-mediated TTP (iTTP) and hereditary or congenital TTP (cTTP). The panel used the Grading of Recommendations Assessment, Development, a… Show more

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Cited by 191 publications
(202 citation statements)
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“…Other groups have also discussed the importance of FFP infusion 26‐28 . Recently, International Society on Thrombosis and Haemostasis (ISTH) has published ISTH Guidelines for Treatment of Thrombotic Thrombocytopenic Purpura in 2020 29 . This guidelines reported that prophylactic FFP infusions may prevent maternal and infant mortality in patients with cTTP during pregnancy without moderate to high‐certainty evidence.…”
Section: Discussionmentioning
confidence: 99%
See 2 more Smart Citations
“…Other groups have also discussed the importance of FFP infusion 26‐28 . Recently, International Society on Thrombosis and Haemostasis (ISTH) has published ISTH Guidelines for Treatment of Thrombotic Thrombocytopenic Purpura in 2020 29 . This guidelines reported that prophylactic FFP infusions may prevent maternal and infant mortality in patients with cTTP during pregnancy without moderate to high‐certainty evidence.…”
Section: Discussionmentioning
confidence: 99%
“…Here, based on our experience, previous studies and ISTH guidelines, we propose a strategy to manage pregnancies in patients with cTTP 26‐29 . Prophylactic FFP infusion at a dosage of at least 5 mL/kg/wk should be started when pregnancy is confirmed.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…F I G U R E 1 Therapeutic strategy of iTTP, adapted from previous work. 9,19,24 ADAMTS13 activity availability still requires several days in most countries, and a probabilistic approach is usually needed to initiate an optimal therapy immediately. In centers where ADAMTS13 activity is available immediately, the full treatment associating TPE, immunosuppression, and caplacizumab should be started as soon as severe ADAMTS13 deficiency (activity < 10%) is documented.…”
Section: Is Caplacizumab Cost-effective?mentioning
confidence: 99%
“…[5][6][7] These thrombi in large or small vessels in the brain may cause these neurological signs and symptoms ranging from headache, forgetfulness, numbness, and weakness of the extremities, and confusion to more severe forms such as seizure, stroke, coma, and death. Therefore, early recognition and prompt treatments such as TPE, 12,23,26 steroids, and caplacizumab (an anti-VWF nanobody) [27][28][29] are important as these microthrombi can lead to permanent brain damage leading to high morbidity and mortality. Rituximab (an anti-CD20 monoclonal antibody) helps suppress antibody production, thus reducing relapses in these patients.…”
Section: Introductionmentioning
confidence: 99%