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Article: Extracellular Vesicles in Sickle Cell Disease: A Promising Tool.

Lamarre, Yann / Nader, Elie / Connes, Philippe / Romana, Marc / Garnier, Yohann

2022 Volume 9, Issue 9

Abstract: Sickle cell disease (SCD) is the most common hemoglobinopathy worldwide. It is characterized by an impairment of shear stress-mediated vasodilation, a pro-coagulant, and a pro-adhesive state orchestrated among others by the depletion of the vasodilator ... ...

Abstract	Sickle cell disease (SCD) is the most common hemoglobinopathy worldwide. It is characterized by an impairment of shear stress-mediated vasodilation, a pro-coagulant, and a pro-adhesive state orchestrated among others by the depletion of the vasodilator nitric oxide, by the increased phosphatidylserine exposure and tissue factor expression, and by the increased interactions of erythrocytes with endothelial cells that mediate the overexpression of adhesion molecules such as VCAM-1, respectively. Extracellular vesicles (EVs) have been shown to be novel actors involved in SCD pathophysiological processes. Medium-sized EVs, also called microparticles, which exhibit increased plasma levels in this pathology, were shown to induce the activation of endothelial cells, thereby increasing neutrophil adhesion, a key process potentially leading to the main complication associated with SCD, vaso-occlusive crises (VOCs). Small-sized EVs, also named exosomes, which have also been reported to be overrepresented in SCD, were shown to potentiate interactions between erythrocytes and platelets, and to trigger endothelial monolayer disruption, two processes also known to favor the occurrence of VOCs. In this review we provide an overview of the current knowledge about EVs concentration and role in SCD.
Language	English
Publishing date	2022-09-05
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2746191-9
ISSN	2306-5354
ISSN	2306-5354
DOI	10.3390/bioengineering9090439
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Vasculopathy in Sickle Cell Disease: From Red Blood Cell Sickling to Vascular Dysfunction.

Nader, Elie / Conran, Nicola / Romana, Marc / Connes, Philippe

Comprehensive Physiology

2021 Volume 11, Issue 2, Page(s) 1785–1803

Abstract: Sickle cell disease (SCD) is a hereditary disorder that leads to the production of an abnormal hemoglobin, hemoglobin S (HbS). HbS polymerizes in deoxygenated conditions, which can prompt red blood cell (RBC) sickling and leaves the RBCs more rigid, ... ...

Abstract	Sickle cell disease (SCD) is a hereditary disorder that leads to the production of an abnormal hemoglobin, hemoglobin S (HbS). HbS polymerizes in deoxygenated conditions, which can prompt red blood cell (RBC) sickling and leaves the RBCs more rigid, fragile, and prone to hemolysis. SCD patients suffer from a plethora of complications, ranging from acute complications, such as characteristic, frequent, and debilitating vaso-occlusive episodes to chronic organ damage. While RBC sickling is the primary event at the origin of vaso-occlusive processes, other factors that can further increase RBC transit times in the microcirculation may also be required to precipitate vaso-occlusive processes. The adhesion of RBC and leukocytes to activated endothelium and the formation of heterocellular aggregates, as well as increased blood viscosity, are among the mechanisms involved in slowing the progress of RBCs in deoxygenated vascular areas, favoring RBC sickling and promoting vascular occlusion. Chronic inflammatory processes and oxidative stress, which are perpetuated by hemolytic events and ischemia-reperfusion injury, result in this pan cellular activation and some acute events, such as stroke and acute chest syndrome, as well as chronic end-organ damage. Furthermore, impaired vasodilation and vasomotor hyperresponsiveness in SCD also contribute to vaso-occlusive processes. Treating SCD as a vascular disease in addition to its hematological perspective, the present article looks at the interplay between abnormal RBC physiology/integrity, vascular dysfunction and clinical severity in SCD, and discusses existing therapies and novel drugs in development that may ameliorate vascular complications in the disease. © 2021 American Physiological Society. Compr Physiol 11:1785-1803, 2021.
MeSH term(s)	Anemia, Sickle Cell/complications ; Erythrocytes ; Hemoglobin, Sickle ; Humans ; Leukocytes ; Vascular Diseases/etiology
Chemical Substances	Hemoglobin, Sickle
Language	English
Publishing date	2021-04-01
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ISSN	2040-4603
ISSN (online)	2040-4603
DOI	10.1002/cphy.c200024
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Article: Extracellular Vesicles in Sickle Cell Disease: Plasma Concentration, Blood Cell Types Origin Distribution and Biological Properties.

Nader, Elie / Garnier, Yohann / Connes, Philippe / Romana, Marc

Frontiers in medicine

2021 Volume 8, Page(s) 728693

Abstract: Prototype of monogenic disorder, sickle cell disease (SCD) is caused by a unique single mutation in the β-globin gene, leading to the production of the abnormal hemoglobin S (HbS). HbS polymerization in deoxygenated condition induces the sickling of red ... ...

Abstract	Prototype of monogenic disorder, sickle cell disease (SCD) is caused by a unique single mutation in the β-globin gene, leading to the production of the abnormal hemoglobin S (HbS). HbS polymerization in deoxygenated condition induces the sickling of red blood cells (RBCs), which become less deformable and more fragile, and thus prone to lysis. In addition to anemia, SCD patients may exhibit a plethora of clinical manifestations ranging from acute complications such as the frequent and debilitating painful vaso-occlusive crisis to chronic end organ damages. Several interrelated pathophysiological processes have been described, including impaired blood rheology, increased blood cell adhesion, coagulation, inflammation and enhanced oxidative stress among others. During the last two decades, it has been shown that extracellular vesicles (EVs), defined as cell-derived anucleated particles delimited by a lipid bilayer, and comprising small EVs (sEVs) and medium/large EVs (m/lEVs); are not only biomarkers but also subcellular actors in SCD pathophysiology. Plasma concentration of m/lEVs, originated mainly from RBCs and platelets (PLTs) but also from the other blood cell types, is higher in SCD patients than in healthy controls. The concentration and the density of externalized phosphatidylserine of those released from RBCs may vary according to clinical status (crisis vs. steady state) and treatment (hydroxyurea). Besides their procoagulant properties initially described, RBC-m/lEVs may promote inflammation through their effects on monocytes/macrophages and endothelial cells. Although less intensely studied, sEVs plasma concentration is increased in SCD and these EVs may cause endothelial damages. In addition, sEVs released from activated PLTs trigger PLT-neutrophil aggregation involved in lung vaso-occlusion in sickle mice. Altogether, these data clearly indicate that EVs are both biomarkers and bio-effectors in SCD, which deserve further studies.
Language	English
Publishing date	2021-08-20
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2775999-4
ISSN	2296-858X
ISSN	2296-858X
DOI	10.3389/fmed.2021.728693
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Article ; Online: Extracellular Vesicles in Sickle Cell Disease

Elie Nader / Yohann Garnier / Philippe Connes / Marc Romana

Frontiers in Medicine, Vol

Plasma Concentration, Blood Cell Types Origin Distribution and Biological Properties

2021 Volume 8

Abstract	Prototype of monogenic disorder, sickle cell disease (SCD) is caused by a unique single mutation in the β-globin gene, leading to the production of the abnormal hemoglobin S (HbS). HbS polymerization in deoxygenated condition induces the sickling of red blood cells (RBCs), which become less deformable and more fragile, and thus prone to lysis. In addition to anemia, SCD patients may exhibit a plethora of clinical manifestations ranging from acute complications such as the frequent and debilitating painful vaso-occlusive crisis to chronic end organ damages. Several interrelated pathophysiological processes have been described, including impaired blood rheology, increased blood cell adhesion, coagulation, inflammation and enhanced oxidative stress among others. During the last two decades, it has been shown that extracellular vesicles (EVs), defined as cell-derived anucleated particles delimited by a lipid bilayer, and comprising small EVs (sEVs) and medium/large EVs (m/lEVs); are not only biomarkers but also subcellular actors in SCD pathophysiology. Plasma concentration of m/lEVs, originated mainly from RBCs and platelets (PLTs) but also from the other blood cell types, is higher in SCD patients than in healthy controls. The concentration and the density of externalized phosphatidylserine of those released from RBCs may vary according to clinical status (crisis vs. steady state) and treatment (hydroxyurea). Besides their procoagulant properties initially described, RBC-m/lEVs may promote inflammation through their effects on monocytes/macrophages and endothelial cells. Although less intensely studied, sEVs plasma concentration is increased in SCD and these EVs may cause endothelial damages. In addition, sEVs released from activated PLTs trigger PLT-neutrophil aggregation involved in lung vaso-occlusion in sickle mice. Altogether, these data clearly indicate that EVs are both biomarkers and bio-effectors in SCD, which deserve further studies.
Keywords	extracellular vesicles ; sickle cell disease ; inflammation ; coagulation ; oxidative stress ; endothelial dysfunction ; Medicine (General) ; R5-920
Subject code	610
Language	English
Publishing date	2021-08-01T00:00:00Z
Publisher	Frontiers Media S.A.
Document type	Article ; Online
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Article ; Online: The Red Blood Cell-Inflammation Vicious Circle in Sickle Cell Disease.

Nader, Elie / Romana, Marc / Connes, Philippe

Frontiers in immunology

2020 Volume 11, Page(s) 454

Abstract: Sickle cell disease (SCD) is a genetic disease caused by a single mutation in the β-globin gene, leading to the production of an abnormal hemoglobin called hemoglobin S (HbS), which polymerizes under deoxygenation, and induces the sickling of red blood ... ...

Abstract	Sickle cell disease (SCD) is a genetic disease caused by a single mutation in the β-globin gene, leading to the production of an abnormal hemoglobin called hemoglobin S (HbS), which polymerizes under deoxygenation, and induces the sickling of red blood cells (RBCs). Sickled RBCs are very fragile and rigid, and patients consequently become anemic and develop frequent and recurrent vaso-occlusive crises. However, it is now evident that SCD is not only a RBC rheological disease. Accumulating evidence shows that SCD is also characterized by the presence of chronic inflammation and oxidative stress, participating in the development of chronic vasculopathy and several chronic complications. The accumulation of hemoglobin and heme in the plasma, as a consequence of enhanced intravascular hemolysis, decreases nitric oxide bioavailability and enhances the production of reactive oxygen species (ROS). Heme and hemoglobin also represent erythrocytic danger-associated molecular pattern molecules (eDAMPs), which may activate endothelial inflammation through TLR-4 signaling and promote the development of complications, such as acute chest syndrome. It is also suspected that heme may activate the innate immune complement system and stimulate neutrophils to release neutrophil extracellular traps. A large amount of microparticles (MPs) from various cellular origins (platelets, RBCs, white blood cells, endothelial cells) is also released into the plasma of SCD patients and participate in the inflammation and oxidative stress in SCD. In turn, this pro-inflammatory and oxidative stress environment further alters the RBC properties. Increased pro-inflammatory cytokine concentrations promote the activation of RBC NADPH oxidase and, thus, raise the production of intra-erythrocyte ROS. Such enhanced oxidative stress causes deleterious damage to the RBC membrane and further alters the deformability of the cells, modifying their aggregation properties. These RBC rheological alterations have been shown to be associated to specific SCD complications, such as leg ulcers, priapism, and glomerulopathy. Moreover, RBCs positive for the Duffy antigen receptor for chemokines may be very sensitive to various inflammatory molecules that promote RBC dehydration and increase RBC adhesiveness to the vascular wall. In summary, SCD is characterized by a vicious circle between abnormal RBC rheology and inflammation, which modulates the clinical severity of patients.
MeSH term(s)	Anemia, Sickle Cell/immunology ; Animals ; Duffy Blood-Group System/immunology ; Erythrocytes/immunology ; Extracellular Traps/physiology ; Hemoglobin, Sickle/genetics ; Hemoglobin, Sickle/metabolism ; Hemolysis ; Humans ; Inflammation ; Mice ; Oxidative Stress ; Reactive Oxygen Species/metabolism ; Receptors, Cell Surface/immunology
Chemical Substances	ACKR1 protein, human ; Duffy Blood-Group System ; Hemoglobin, Sickle ; Reactive Oxygen Species ; Receptors, Cell Surface
Language	English
Publishing date	2020-03-13
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2020.00454
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Article: Pathogenesis and immunopathology of paraneoplastic disorders.

Quinot, Valérie / Höftberger, Romana

Handbook of clinical neurology

2024 Volume 200, Page(s) 33–54

Abstract: ... Neuropathologic hallmarks of PNS associated with antibodies directed against intracellular epitopes are ...

Abstract	Paraneoplastic neurologic syndromes (PNS) represent a rare group of immune-mediated complications associated with an underlying tumor. Ectopic protein expression in neoplastic cells or an aberrant immune regulation in the course of hematooncologic diseases or thymomas trigger an autoimmune response that may affect any part of the central and/or peripheral nervous system. Recent advances in drug therapies as well as novel animal models and neuropathologic studies have led to further insights on the immune pathomechanisms of PNS. Although the syndromes share common paths in pathogenesis, they may differ in the disease course, prognosis, and therapy targets, depending on the localization and type of antibody epitope. Neuropathologic hallmarks of PNS associated with antibodies directed against intracellular epitopes are characterized by T cell-dominated inflammation, reactive gliosis including microglial nodules, and neuronal degeneration. By contrast, the neuropathology of cell surface antibody-mediated PNS strongly depends on the targeted antigen and varies from B cell/plasma cell-dominated inflammation and well-preserved neurons together with a reduced expression of the target antigen in anti-NMDAR encephalitis to irreversible Purkinje cell loss in anti-P/Q-type VGCC antibody-associated paraneoplastic cerebellar degeneration. The understanding of different pathomechanisms in PNS is important because they strongly correspond with therapy response and prognosis, and should guide treatment decisions.
MeSH term(s)	Animals ; Humans ; Autoantibodies ; Paraneoplastic Syndromes, Nervous System ; Nervous System Diseases/complications ; Neoplasms/complications ; Inflammation
Chemical Substances	Autoantibodies
Language	English
Publishing date	2024-02-21
Publishing country	Netherlands
Document type	Review ; Journal Article
ISSN	0072-9752
ISSN	0072-9752
DOI	10.1016/B978-0-12-823912-4.00027-X
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Article ; Online: Terminologies regarding sickle cell retinopathy and maculopathy.

Beral, Laurence / Romana, Marc / Lemonne, Nathalie / David, Thierry / Connes, Philippe

Clinical hemorheology and microcirculation

2019 Volume 71, Issue 1, Page(s) 1–2

MeSH term(s)	Anemia, Sickle Cell ; Humans ; Retinal Diseases ; Rheology
Language	English
Publishing date	2019-02-13
Publishing country	Netherlands
Document type	Letter ; Comment
ZDB-ID	1381750-4
ISSN	1875-8622 ; 1386-0291
ISSN (online)	1875-8622
ISSN	1386-0291
DOI	10.3233/CH-190552
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Article ; Online: Pain Control for Sickle Cell Crisis, a Novel Approach? A Retrospective Study.

Rollé, Amélie / Vidal, Elsa / Laguette, Pierre / Garnier, Yohann / Delta, Delphine / Martino, Frédéric / Portecop, Patrick / Etienne-Julan, Maryse / Piednoir, Pascale / De Jong, Audrey / Romana, Marc / Bernit, Emmanuelle

Medicina (Kaunas, Lithuania)

2023 Volume 59, Issue 12

Abstract: Background and ... ...

Abstract	Background and Objectives
MeSH term(s)	Adult ; Female ; Humans ; Pain Management/methods ; Retrospective Studies ; Analgesics, Opioid/therapeutic use ; Volatile Organic Compounds ; Pain/drug therapy ; Pain/etiology ; Analgesics/therapeutic use ; Anemia, Sickle Cell/complications
Chemical Substances	Analgesics, Opioid ; Volatile Organic Compounds ; Analgesics
Language	English
Publishing date	2023-12-18
Publishing country	Switzerland
Document type	Systematic Review ; Journal Article
ZDB-ID	2188113-3
ISSN	1648-9144 ; 1010-660X
ISSN (online)	1648-9144
ISSN	1010-660X
DOI	10.3390/medicina59122196
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Article ; Online: Toward a Conversational Agent to Support the Self-Management of Adults and Young Adults With Sickle Cell Disease: Usability and Usefulness Study.

Issom, David-Zacharie / Hardy-Dessources, Marie-Dominique / Romana, Marc / Hartvigsen, Gunnar / Lovis, Christian

Frontiers in digital health

2021 Volume 3, Page(s) 600333

Abstract: Sickle cell disease (SCD) is the most common genetic blood disorder in the world and affects millions of people. With aging, patients encounter an increasing number of comorbidities that can be acute, chronic, and potentially lethal (e.g., pain, multiple ...

Abstract	Sickle cell disease (SCD) is the most common genetic blood disorder in the world and affects millions of people. With aging, patients encounter an increasing number of comorbidities that can be acute, chronic, and potentially lethal (e.g., pain, multiple organ damages, lung disease). Comprehensive and preventive care for adults with SCD faces disparities (e.g., shortage of well-trained providers). Consequently, many patients do not receive adequate treatment, as outlined by evidence-based guidelines, and suffer from mistrust, stigmatization or neglect. Thus, adult patients often avoid necessary care, seek treatment only as a last resort, and rely on self-management to maintain control over the course of the disease. Hopefully, self-management positively impacts health outcomes. However, few patients possess the required skills (e.g., disease-specific knowledge, self-efficacy), and many lack motivation for effective self-care. Health coaching has emerged as a new approach to enhance patients' self-management and support health behavior changes. Recent studies have demonstrated that conversational agents (chatbots) could effectively support chronic patients' self-management needs, improve self-efficacy, encourage behavior changes, and reduce disease-severity. To date, the use of chatbots to support SCD self-management remains largely under-researched. Consequently, we developed a high-fidelity prototype of a fully automated health coaching chatbot, following patient-important requirements and preferences collected during our previous work. We recruited a small convenience sample of adults with SCD to examine the usability and perceived usefulness of the system. Participants completed a post-test survey using the System Usability Scale and the Usefulness Scale for Patient Information Material questionnaire. Thirty-three patients participated. The majority (64%) was affected by the most clinically severe SCD genotypes (Hb SS, HbSβ0). Most participants (94%) rated the chatbots as easy and fun to use, while 88% perceived it as useful support for patient empowerment. In the qualitative phase, 72% of participants expressed their enthusiasm using the chatbot, and 82% emphasized its ability to improve their knowledge about self-management. Findings suggest that chatbots could be used to promote the acquisition of recommended health behaviors and self-care practices related to the prevention of the main symptoms of SCD. Further work is needed to refine the system, and to assess clinical validity.
Language	English
Publishing date	2021-01-29
Publishing country	Switzerland
Document type	Journal Article
ISSN	2673-253X
ISSN (online)	2673-253X
DOI	10.3389/fdgth.2021.600333
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Article ; Online: Microparticles in sickle cell disease.

Romana, Marc / Connes, Philippe / Key, Nigel S

Clinical hemorheology and microcirculation

2018 Volume 68, Issue 2-3, Page(s) 319–329

Abstract: Several pathophysiological pathways in sickle cell disease (SCD), the most prevalent hemoglobinopathy worldwide, result in activation of circulating blood cells and the release of submicron vesicles, so-called microparticles (MPs). MPs are candidate ... ...

Abstract	Several pathophysiological pathways in sickle cell disease (SCD), the most prevalent hemoglobinopathy worldwide, result in activation of circulating blood cells and the release of submicron vesicles, so-called microparticles (MPs). MPs are candidate biomarkers in vascular disease that exhibit functional biological properties. Compared to healthy individuals, higher level of plasma MPs, mostly derived from platelets and red blood cells (RBC), has been repeatedly observed in SCD patients in their steady-state condition. In contrast, conflicting results have been obtained on the impact of SCD complications and hydroxyurea treatment on circulating MP concentrations, largely due to non-standardized pre- and analytical procedures. Several factors responsible for the increased release of MPs by RBC have been identified in SCD such as sickling/unsickling, oxidative stress and abnormal activity of RBC acid sphingomyelinase. Besides their well-known pro-coagulant effect, sickle RBC-derived MPs produced ex vivo can induce ROS production by endothelial cells and promote a pro-inflammatory and pro-adhesive phenotype that may lead to renal occlusion in SCD mice. However, the functional properties of circulating MPs in human sickle cell disease remain to be studied and fully characterized.
MeSH term(s)	Anemia, Sickle Cell/blood ; Anemia, Sickle Cell/physiopathology ; Cell-Derived Microparticles/metabolism ; Erythrocytes/metabolism ; Humans
Language	English
Publishing date	2018
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	1381750-4
ISSN	1875-8622 ; 1386-0291
ISSN (online)	1875-8622
ISSN	1386-0291
DOI	10.3233/CH-189014
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