Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors

Upreti, Harshvardhan; Kasmani, Jamil; Dane, Kathryn; Braunstein, Evan M.; Streiff, Michael B.; Shanbhag, Satish; Moliterno, Alison R.; Sperati, C. John; Gottesman, Rebecca F.; Brodsky, Robert A.; Kickler, Thomas S.; Chaturvedi, Shruti

doi:10.1182/blood.2019001056

Cited by 65 publications

(76 citation statements)

References 47 publications

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“…[42][43][44] Also in survivors of an initial acute episode of immune-mediated TTP, reduced ADAMTS13 activity ( 70%) was found to be a risk factor for subsequent ischemic strokes. 45 At the end of 2019, physicians at 44 sites in 19 countries (on four continents) followed up participants in the Hereditary TTP Registry which has participant information and informed consent documents available in 17 languages. This makes the Hereditary TTP Registry a large international but at the same time a difficult and demanding project, possibly an inevitable aspect when aiming at meaningful patient numbers in an ultra-rare disease.…”

Section: The Hereditary Ttp Registrymentioning

confidence: 99%

Insights from the Hereditary Thrombotic Thrombocytopenic Purpura Registry: Discussion of Key Findings Based on Individual Cases from Switzerland

et al. 2020

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The Hereditary TTP Registry is an international cohort study for patients with a confirmed or suspected diagnosis of hereditary thrombotic thrombocytopenic purpura (hTTP) and their family members. Hereditary TTP is an ultra-rare blood disorder (prevalence of ∼1–2 cases per million), the result of autosomal-recessively inherited congenital ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) deficiency (ADAMTS13 activity <10% of the normal), and associated with yet many unanswered questions. Until December 2017, the Hereditary TTP Registry had enrolled 123 confirmed hTTP patients. Their median age at disease onset was 4.5 years (range: 0–70) and at clinical diagnosis 16.7 years (range: 0–69), a difference that highlights the existing awareness gap in recognizing hTTP. The systematic collection of clinical data of individual patients revealed their substantial baseline comorbidities, as a consequence of recurring TTP episodes in the past. Most notable was the high proportion of patients having suffered from premature arterial thrombotic events, mainly transient ischemic attacks, ischemic strokes, and to a lesser extent myocardial infarctions. At 40 to 50 years of age and above, more than 50% of patients had suffered from at least one such event, and many had experienced arterial thrombotic events despite regular plasma infusions every 2 to 3 weeks that supplements the missing plasma ADAMTS13. The article by van Dorland et al. (Haematologica 2019;104(10):2107–2115) and the ongoing Hereditary TTP Registry cohort study were recognized with the Günter Landbeck Excellence Award at the 50th Hemophilia Symposium in Hamburg in November 2019, the reason to present the Hereditary TTP Registry in more detail here.

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Section: The Hereditary Ttp Registrymentioning

confidence: 99%

Insights from the Hereditary Thrombotic Thrombocytopenic Purpura Registry: Discussion of Key Findings Based on Individual Cases from Switzerland

et al. 2020

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“…There is emerging evidence that low-normal or reduced ADAMTS13 activity during remission is associated with an increased risk for ischemic stroke outside of iTTP relapse. 6 Based on the existing evidence, we believe that assessment of ADAMTS13 activity should be performed regularly for a minimum of 2 years after an acute iTTP episode; long-term or even lifelong assessment is preferable.…”

Section: Demographic Risk Factors Of Ittp Relapsementioning

confidence: 99%

Evidence-Based Minireview: Laboratory surveillance of immune-mediated thrombotic thrombocytopenic purpura

Hovinga

2020

Hematology

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“…4 Indeed, a recent study showed that stroke after iTTP is associated with persistently low ADAMTS13 activity during iTTP remission independent of age. 5 Previously, large population-based studies have demonstrated an association between lower ADAMTS13 activity with myocardial infarction and stroke. 6,7 Remission ADAMTS13 activity after recovery from acute iTTP is an attractive candidate as a biomarker and therapeutic target to improve long-term outcomes in all patients with iTTP.…”

Section: University Of Pennsylvaniamentioning

confidence: 99%

Different strokes for older folks (with TTP)

Chaturvedi

Cuker

2019

Blood

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Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors

Abstract: This study shows that the increased occurrence of stroke in thrombotic thrombocytopenic purpura (TTP) during remission is associated with low ADAMTS13 values.

Cited by 65 publications

References 47 publications

Insights from the Hereditary Thrombotic Thrombocytopenic Purpura Registry: Discussion of Key Findings Based on Individual Cases from Switzerland

Insights from the Hereditary Thrombotic Thrombocytopenic Purpura Registry: Discussion of Key Findings Based on Individual Cases from Switzerland

Evidence-Based Minireview: Laboratory surveillance of immune-mediated thrombotic thrombocytopenic purpura

Different strokes for older folks (with TTP)

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