OC 42.2 - Incidence and Risk Factors of Major Adverse Cardiovascular Events in Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura During Remission

Monday, June 26, 2023

15:00 – 15:15 ET

Room: 710A

Session Available As: On-Demand

Presenting Author(s)

Pasquale Agosti, MD, PhD

Università degli Studi di Milano, Department of Pathophysiology and Transplantation and Fondazione Luigi Villa, Milan, Italy
Milano, Lombardia, Italy

Co-Author(s)

FB

Federico Boggio

Resident in Internal Medicine
Università degli Studi di Milano
Milan, Lombardia, Italy
PD

Pasqualina De Leo

Biologist
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
Addolorata Truma, MD

Resident doctor in Internal Medicine
Università degli Studi di Milano, Department of Pathophysiology and Transplantation
Milano, Lombardia, Italy
Juri Alessandro Giannotta, MD

Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milano, Lombardia, Italy
Andrea Artoni, MD

MD
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milano, Lombardia, Italy
BF

Barbara Ferrari

MD
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
IM

Ilaria Mancini, PhD

Post-Doc Research Fellow
Università degli Studi di Milano, Department of Pathophysiology and Transplantation and Fondazione Luigi Villa, Milan, Italy
Milan, Lombardia, Italy
Flora Peyvandi, MD, PhD (she/her/hers)

Director
IRCCS Ca' Granda Maggiore Hospital Foundation, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milano, Italy; Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milano, Italy
Milano, Lombardia, Italy

Background: In patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP), an increased risk of major adverse cardiovascular events (MACE) during remission has been reported. However, data on MACE prevalence and related risk factors are scarce.

Aims: To evaluate the rate of MACE during remission in Italian iTTP survivors and related risk factors.

Methods: In this cohort study, we enrolled patients from the Milan TTP Registry surviving the first acute episode of iTTP and followed-up at the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center (Milan) for at least six months. Patients with history of ischemic stroke (IS) or acute coronary syndrome (ACS) and lost to follow-up before 2012 were excluded. MACE was defined as occurrence of ACS or IS.

Results: We included 238 patients, of whom 8 (3.4%) had at least one MACE, with a total of 11 MACE, mostly IS (3 ACS, 7 symptomatic IS and 1 silent IS). We found the following age-stratified MACE rates: 5/169 (3%) at ages < 50 years, 2/63 (3.2 %) in patients aged 50-69 years, 1/7 (14.3%) in patients older than 70 years. These rates were consistent with a population of moderate-high cardiovascular risk. Patients presenting with MACE were slightly older than those without, with a higher proportion of males. Regular ADAMTS13 testing during remission was unavailable before MACE, although six patients (75%) showed persistently reduced ADAMTS13 activity later in life (Table 1). Compared with an Italian population study with a similar age and sex distribution, our cohort showed higher IS rates despite a lower prevalence of atherogenic risk factors (Table 2).

Conclusion(s): In our cohort of Italian iTTP patients, we found a MACE rate of 3.4% during remission. ACS and IS rates were similar and 3-fold higher than in an Italian population, respectively, supporting a possible major role of microvessels dysfunction over atherosclerosis progression in iTTP.

Learning Objectives:

Upon completion, participant will be able to describe and explain the incidence of major adverse cardiovascular events during remission In patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP).
Upon completion, participant will be able to predict the risk of major adverse cardiovascular events during remission In patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP).
Upon completion, participant will be able to identify the risk factors related to major adverse cardiovascular events occurring during remission In patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP).