Cerebral Small Vessel Disease in Immune‐Mediated Thrombotic Thrombocytopenic Purpura Patients During the Acute Phase and Disease Remission
4 affiliations
- 1Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Milan Italy
- 2Neuroradiology Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy
- 3Department of Pathophysiology and Transplantation Università Degli Studi di Milano Milan Italy
- 4Medical Physics Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy
Abstract
Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a life‐threatening thrombotic microangiopathy characterized by acute neurological manifestations and long‐term sequelae related to microvascular brain injury. Cerebral small vessel disease (cSVD), detectable on MRI through lacunes, microbleeds, white matter hyperintensities, and enlarged perivascular spaces, is a risk factor for stroke and cognitive decline, but its prevalence and evolution in iTTP are unknown. We conducted a prospective study of consecutive patients with acute iTTP and age‐matched controls to assess the prevalence and one‐year progression of cSVD. Brain MRI was performed at enrollment and repeated after 12–18 months in clinical remission. cSVD burden was quantified using a standardized 0–4 score based on four MRI markers. Twenty‐five patients (median age 56 years; 60% women) were enrolled. Acute focal ischemic lesions were detected in 13 (52%). cSVD was present in 48% of patients versus 27% of controls, while moderate burden (score ≥ 2) occurred in 24% versus none. Ordinal logistic regression showed an association between iTTP and higher cSVD scores (OR 3.19, 95% CI 1.00–10.14), independent of traditional cardiovascular risk factors. Follow‐up MRI was available in 22 patients (88%). In paired analyses, cSVD prevalence increased from 50% to 64%, moderate‐to‐high burden from 27% to 41%, and six patients (27%) showed score progression. At 1 year, patients had higher odds of greater cSVD burden than controls (age‐ and sex‐adjusted ordinal OR 7.00, 95% CI 1.65–29.74). These findings indicate that cSVD is common during acute iTTP and progresses during the first year despite hematological remission.
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BibTeX
@article{truma2026cerebral,
title = {{Cerebral Small Vessel Disease in Immune‐Mediated Thrombotic Thrombocytopenic Purpura Patients During the Acute Phase and Disease Remission}},
author = {Addolorata Truma and Francesco Maria Lo Russo and Giorgio Conte and Ilaria Mancini and Andrea Artoni and Juri Alessandro Giannotta and Barbara Ferrari and Pasquale Agosti and Maria Abbattista and Matteo Gagliardi and Eleonora Piccin and Marco Stroppi and Fabio Maria Triulzi and Flora Peyvandi},
journal = {American Journal of Hematology},
year = {2026},
month = sep,
doi = {10.1002/ajh.70506},
url = {https://doi.org/10.1002/ajh.70506},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Cerebral Small Vessel Disease in Immune‐Mediated Thrombotic Thrombocytopenic Purpura Patients During the Acute Phase and Disease Remission
AU - Addolorata Truma
AU - Francesco Maria Lo Russo
AU - Giorgio Conte
AU - Ilaria Mancini
AU - Andrea Artoni
AU - Juri Alessandro Giannotta
AU - Barbara Ferrari
AU - Pasquale Agosti
AU - Maria Abbattista
AU - Matteo Gagliardi
AU - Eleonora Piccin
AU - Marco Stroppi
AU - Fabio Maria Triulzi
AU - Flora Peyvandi
PY - 2026
DA - 2026/09/22
JO - American Journal of Hematology
DO - 10.1002/ajh.70506
UR - https://doi.org/10.1002/ajh.70506
AB - Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a life‐threatening thrombotic microangiopathy characterized by acute neurological manifestations and long‐term sequelae related to microvascular brain injury. Cerebral small vessel disease (cSVD), detectable on MRI through lacunes, microbleeds, white matter hyperintensities, and enlarged perivascular spaces, is a risk factor for stroke and cognitive decline, but its prevalence and evolution in iTTP are unknown. We conducted a prospective study of consecutive patients with acute iTTP and age‐matched controls to assess the prevalence and one‐year progression of cSVD. Brain MRI was performed at enrollment and repeated after 12–18 months in clinical remission. cSVD burden was quantified using a standardized 0–4 score based on four MRI markers. Twenty‐five patients (median age 56 years; 60% women) were enrolled. Acute focal ischemic lesions were detected in 13 (52%). cSVD was present in 48% of patients versus 27% of controls, while moderate burden (score ≥ 2) occurred in 24% versus none. Ordinal logistic regression showed an association between iTTP and higher cSVD scores (OR 3.19, 95% CI 1.00–10.14), independent of traditional cardiovascular risk factors. Follow‐up MRI was available in 22 patients (88%). In paired analyses, cSVD prevalence increased from 50% to 64%, moderate‐to‐high burden from 27% to 41%, and six patients (27%) showed score progression. At 1 year, patients had higher odds of greater cSVD burden than controls (age‐ and sex‐adjusted ordinal OR 7.00, 95% CI 1.65–29.74). These findings indicate that cSVD is common during acute iTTP and progresses during the first year despite hematological remission.
SN - 0361-8609
SN - 1096-8652
ER -