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Guest Editor

Department of Pathophysiology and Transplantation, and Fondazione Luigi Villa, Università degli Studi di Milano, 20122 Milan, Italy
Interests: thrombotic thrombocytopenic purpura; thrombotic microangiopathies; ADAMTS13; laboratory ADAMTS13 testing; genetics of hemostasis and thrombotic disorders

Dr. Andrea Artoni

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Guest Editor

Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, 20122 Milan, Italy
Interests: thrombotic thrombocytopenic purpura; thrombotic microangiopathies; coagulation; thrombosis; haemostasis; platelets; platelet aggregation; hematology; hemostasis; blood coagulation; platelet activation; pulmonary embolism

Special Issue Information

Dear Colleagues,

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy caused by the congenital or acquired severe deficiency of the von Willebrand factor cleaving protease, ADAMTS13. Acute TTP is a medical emergency requiring a fast differential diagnosis and immediate treatment, which is often challenging due to the overlap of clinical and laboratory features with other thrombotic microangiopathies. Diagnostic and therapeutic advancements in the last decade, including the increased availability of ADAMTS13 testing and the development of caplacizumab, the first targeted therapy for acquired TTP, have significantly improved the management of acute TTP and reduced the burden of such short-term outcomes as mortality and exacerbation of acute disease. At the same time, new clinical challenges have emerged, to mention one addressing the burden of long-term complications of TTP and cardiovascular comorbidities in TTP patients in remission.

This Special Issue will cover new diagnostic and therapeutic aspects of TTP care, with the scope of advancing our knowledge and clinical intervention to address the current challenges of TTP management. Original, review, and guidance/guidelines papers are welcome.

Dr. Ilaria Mancini
Dr. Andrea Artoni
Guest Editors

Manuscript Submission Information

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Keywords

thrombotic thrombocytopenic purpura (TTP)
ADAMTS13
ADAMTS13 testing
differential diagnosis of thrombotic microangiopathy
disease awareness
TTP therapy
refractoriness
exacerbation
thromboembolic complications
new therapeutic approaches
long-term outcomes
TTP relapse prevention
pregnancy management
management of predisposing conditions/precipitating factors
hospital resources

Published Papers (2 papers)

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Review

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Open AccessReview

Is Endothelial Activation a Critical Event in Thrombotic Thrombocytopenic Purpura?

Raphael Cauchois

Romain Muller

Marie Lagarde

Françoise Dignat-George

Edwige Tellier

and

Gilles Kaplanski

J. Clin. Med. 2023, 12(3), 758; https://doi.org/10.3390/jcm12030758 - 18 Jan 2023

Viewed by 762

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a severe thrombotic microangiopathy. The current pathophysiologic paradigm suggests that the ADAMTS13 deficiency leads to Ultra Large-Von Willebrand Factor multimers accumulation with generation of disseminated microthrombi. Nevertheless, the role of endothelial cells in this pathology remains an issue. In this review, we discuss the various clinical, in vitro and in vivo experimental data that support the important role of the endothelium in this pathology, suggesting that ADAMTS13 deficiency may be a necessary but not sufficient condition to induce TTP. The “second hit” model suggests that in TTP, in addition to ADAMTS13 deficiency, endogenous or exogenous factors induce endothelial activation affecting mainly microvascular cells. This leads to Weibel–Palade bodies degranulation, resulting in UL-VWF accumulation in microcirculation. This endothelial activation seems to be worsened by various amplification loops, such as the complement system, nucleosomes and free heme. Full article

(This article belongs to the Special Issue New Diagnostic and Therapeutic Aspects of Thrombotic Thrombocytopenic Purpura)

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Other

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Open AccessCase Report

Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise

and

J. Clin. Med. 2022, 11(23), 7115; https://doi.org/10.3390/jcm11237115 - 30 Nov 2022

Cited by 1 | Viewed by 680

Abstract

Background: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder caused by an inherited genetic deficiency of ADAMTS13 and affects less than one per million individuals. Patients who are diagnosed with TTP during pregnancy are at increased risk of maternal and fetal complications including fetal demise. We present a case of a 32-year-old G3P0 (gravida 3, para 0) who presented at 20 weeks gestation with a new diagnosis of congenital TTP (cTTP) and fetal demise. Methods: We describe the pathophysiology of pregnancy complications in a patient with cTTP using platelet procoagulant membrane dynamics analysis and quantitative proteomic studies, compared to four pregnant patients with gestational hypertension, four pregnant patients with preeclampsia, and four healthy pregnant controls. Results: The cTTP patient had increased P-selectin, tissue factor expression, annexin-V binding on platelets and neutrophils, and localized thrombin generation, suggestive of hypercoagulability. Among 15 proteins that were upregulated, S100A8 and S100A9 were distinctly overexpressed. Conclusions: There is platelet-neutrophil activation and interaction, platelet hypercoagulability, and proinflammation in our case of cTTP with fetal demise. Full article

(This article belongs to the Special Issue New Diagnostic and Therapeutic Aspects of Thrombotic Thrombocytopenic Purpura)

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