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| Name | Class |
|---|---|
| Istituto Auxologico Italiano | OTHER |
| Centro Cardiologico Monzino | OTHER |
| Politecnico di Milano | OTHER |
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The goal of this interventional, non-pharmacological clinical study is to assess microcirculatory dysfunction in patients with thrombotic thrombocytopenic purpura (TTP), both during the acute phase and in remission, compared with the general population.
Microcirculatory damage will be defined as the presence of at least one abnormal instrumental finding among brain magnetic resonance imaging (MRI), optical coherence tomography angiography (OCT-A), and nailfold videocapillaroscopy.
This is a monocentric, national study including two patient cohorts:
Cohort 1: patients enrolled during the acute phase Cohort 2: patients enrolled during remission Participants will be followed for three years with a total of five study visits.
Researchers will not test any drug but will perform detailed clinical, laboratory, and instrumental assessments to evaluate microvascular involvement over time.
Participants will:
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Acute TTP | Other | Patients enrolled during acute phase (cohort 1), T0= within 15 days from hospital admission. |
|
| Remission TTP | Other | Patients enrolled during clinical remission (cohort 2), T0= check-up visit performed at more than 3 months from clinical response of the previous episode |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Factor VIII (FVIII) | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| Measure | Description | Time Frame |
|---|---|---|
| Prevalence | Prevalence of microcirculation damage in iTTP patients compared to general population | From enrollment to the last visit (after 36 months from Time 0) |
| Measure | Description | Time Frame |
|---|---|---|
| Cerebral microcirculation lesions | Evaluate cerebral microcirculation lesions (via brain MRI) in both acute and remission phases of TTP to monitor organ damage progression over time | From enrollment to the last visit (after 36 months from Time 0) |
| Retinal microcirculation changes |
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Inclusion Criteria:
Exclusion Criteria:
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico | Milan | Milano | 20122 | Italy |
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| Von Willebrand factor (VWF) antigen | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| VWF:RCo | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| VWF-propeptide | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| VWF multimers | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| sP-selectin | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| sCD40L | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| F1+2 | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| VEGF-A | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| VCAM | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| ICAM | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| IL-1β | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| IL-6 | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| PTX3 | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| BlyS | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| Neutrophil elastase and myeloperoxidase (NETs) markers | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| soluble C5b-9 | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
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| C5a | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
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| NFL | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
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| GFAP | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
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| evaluation of thrombus formation with Total Thrombus formation Analysis System (T-TAS) | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
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| Microfluidic Flow Chamber | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
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| Whole exome sequencing - WES | Genetic | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
| Proteomic Analysis | Diagnostic Test | The study is defined as interventional because it includes assessments that are not part of routine clinical practice |
|
Assess retinal microcirculation changes (using OCT and OCT-A) during acute and remission phases of TTP to track organ damage evolution |
| From enrollment to the last visit (after 36 months from Time 0) |
| Peripheral microcirculation | Analyze peripheral microcirculation (with nailfold videocapillaroscopy) in acute and remission phases to evaluate progression of organ involvement in TTP | From enrollment to the last visit (after 36 months from Time 0) |
| Association between cerebral MRI lesions and cognitive or psychological symptoms | Explore the relationship between cerebral MRI lesions and cognitive or psychological symptoms during TTP remission, assessing symptom progression | From enrollment to the last visit (36 months from Time 0) |
| Modification of preclinical biomarkers | Explore the modification of preclinical biomarkers over time at different stage of the disease | From enrollment to the last visit (36 months from Time 0) |
| Associations between preclinical biomarkers and microcirculatory damage | Identify associations between preclinical biomarkers and microcirculatory damage in both acute and remission phases of TTP to predict risk and progression | From enrollment to the last visit (36 months from Time 0) |
| ID | Term |
|---|---|
| D011697 | Purpura, Thrombotic Thrombocytopenic |
| ID | Term |
|---|---|
| D011696 | Purpura, Thrombocytopenic |
| D011693 | Purpura |
| D001778 | Blood Coagulation Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D057049 | Thrombotic Microangiopathies |
| D013921 | Thrombocytopenia |
| D001791 | Blood Platelet Disorders |
| D000095542 | Cytopenia |
| D019851 | Thrombophilia |
| D006470 | Hemorrhage |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D012877 | Skin Manifestations |
| D012816 | Signs and Symptoms |
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| ID | Term |
|---|---|
| D000073359 | Exome Sequencing |
| ID | Term |
|---|---|
| D000073336 | Whole Genome Sequencing |
| D017422 | Sequence Analysis, DNA |
| D017421 | Sequence Analysis |
| D005821 | Genetic Techniques |
| D008919 | Investigative Techniques |
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