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Papers by gonzalo de luna

Blood

Sudden death is 1 of the leading causes of death in adults with sickle cell anemia (SCA) but its ... more Sudden death is 1 of the leading causes of death in adults with sickle cell anemia (SCA) but its etiology remains mostly unknown. Ventricular arrhythmia (VA) carries an increased risk of sudden death; however, its prevalence and determinants in SCA are poorly studied. This study aimed to identify the prevalence and predictors of VA in patients with SCA. From 2019 to 2022, 100 patients with SCA were referred to the physiology department to specifically analyze cardiac function and prospectively included in the DREPACOEUR registry. They underwent a 24-hour electrocardiogram monitoring (24h-Holter), transthoracic echocardiography, and laboratory tests on the same day. The primary end point was the occurrence of VA, defined as sustained or nonsustained ventricular tachycardia (VT), >500 premature ventricular contractions (PVCs) on 24h-Holter, or a recent history of VT ablation. The mean patient age was 46 ± 13 years, and 48% of the patients were male. Overall, VA was observed in 22 (...

Transfusion Clinique et Biologique

Introduction: Because of iron overload complications, thrombosis and infectious predisposition, p... more Introduction: Because of iron overload complications, thrombosis and infectious predisposition, patients with severe forms of thalassemia are likely to be at increased risk of COVID-19 complications. Results: A national survey conducted during the year 2020 across the French reference centers for hemoglobinopathies identified 16 cases of COVID-19 confirmed by RT-PCR in beta-thalassemia patients. Their age ranged from 11 months to 60 years. 15 patients were transfusion dependent and 6 were splenectomized. Concerning iron-overload related complications, none had diabetes or cirrhosis and only one had experienced heart failure. All 4 pediatric patients were pauci-symptomatic during the viral episode. Three patients (41, 49 and 57 years old) developed COVID-19 pneumonia requiring oxygen-therapy without the need for mechanical ventilation. Neutropenia (absolute neutrophils count <0.5 10 9/L) was observed in 2 patients receiving long-term treatment with hydroxycarbamide and deferiprone. No thrombosis event, organ failure or death occurred. All patients recovered. Conclusion: Severity of COVID-19 in this population of early-aged patients appeared increased compared to the general population but remained mild to moderate as already reported in the few series reported in the literature. Occurrence of adverse events related to chronic treatment administered in thalassemia disease may be favored by the infectious episode.

Transfusion Clinique et Biologique, 2021

Frontiers in Neuroscience, 2016

Neurodegenerative diseases affect a significant portion of the aging population. Several lines of... more Neurodegenerative diseases affect a significant portion of the aging population. Several lines of evidence suggest a positive association between environmental exposures, which are common and cumulative in a lifetime, and development of neurodegenerative diseases. Environmental or occupational exposure to manganese (Mn) has been implicated in neurodegeneration due to its ability to induce mitochondrial dysfunction, oxidative stress, and α-synuclein (α-Syn) aggregation. The role of the α-Syn protein visa-vis Mn is controversial, as it seemingly plays a duplicitous role in neuroprotection and neurodegeneration. α-Syn has low affinity for Mn, however an indirect interaction cannot be ruled out. In this review we will examine the current knowledge surrounding the interaction of α-Syn and Mn in neurodegenerative process.

Objetivos: Analizar una serie de pacientes diagnosticados de enfermedad de Kikuchi (EK, linfadeni... more Objetivos: Analizar una serie de pacientes diagnosticados de enfermedad de Kikuchi (EK, linfadenitis histiocítica necrotizante) en nuestro hospital, y describir la asociación con enfermedades autoinmunes sistémicas (EAS) y onco-hematológicas. Métodos: Estudio descriptivo de las características clínicas y epidemiológicos de 18 pacientes con EK, con diagnóstico anatomopatológico. Se revisaron las historias clínicas retrospectivamente desde el 1993 hasta el 2014. Resultados: Durante el estudio, se obtuvo el diagnóstico EK con anatomía patológica de linfadenitis histiocítica necrotizante en 18 pacientes. De ellos, 16/18 (88%) eran caucásicos, 1/18 (6%) africano y 1/18 (6%) asiático. Predominó el sexo femenino con 16/17 (94%). La edad media al diagnóstico fue de 30 años (± 11,64). Clínicamente, 12/18 (65%) presentaron fiebre y todos debutaron con enfermedad ganglionar cervical. Dos pacientes (11%) presentaron adenopatías inguinales. Síntomas como rash o artralgias estuvieron presentes en...

Enfermedades Infecciosas y Microbiología Clínica, 2011

cervantesvirtual.com

La Biblioteca Virtual Miguel de Cervantes, la primera en lengua castellana, es un fondo bibliográ... more La Biblioteca Virtual Miguel de Cervantes, la primera en lengua castellana, es un fondo bibliográfico con obras de Literatura, Historia, Ciencias, etc., de libre acceso. Incluye trabajos de investigación, catálogo en otras lenguas y bibliotecas del mundo.

American Journal of Hematology

American Journal of Hematology

Blood, Dec 7, 2017

Introduction : Vaso-Occlusive Crisis (VOC), the most common manifestation of sickle cell disease ... more Introduction : Vaso-Occlusive Crisis (VOC), the most common manifestation of sickle cell disease (SCD), is the first cause of death, particularly when complicated by an acute chest syndrome (ACS). In the PRESEV1 study, we developed a predictive score of secondary ACS in SCD patient admitted in the hospital for VOC (AUC = 0.840 [95% CI: 0.780 - 0.900]), with a negative predictive value of 98.8% for the low level risk and a positive predictive value of 39.5%. The aim of the PRESEV-2 study is to validate the predictive score of ACS on adults and children with SCD in Europe and Africa. Herein we present preliminary data on 235 adults with a severe VOC included in 11 referral centers from 4 countries. Patients and Methods: We conduct an international multicenter prospective observational study. The primary outcome measure is the occurrence of an ACS. The ACS is defined as a new auscultatory abnormality (crepitation or bronchial breathing) OR the association of a new radiologic infiltrate AND chest pain or decreased breath sounds. Secondary outcome measures are length of hospitalization, transfusion, morphine consumption, mortality and hospitalization in intensive care unit. Inclusion criteria are: SS or S-b0 thalassemia patients, VOC admitted at the emergency unit (VOC is defined as pain or tenderness affecting at least one part of the body not attributable to other causes) that required hospitalization and opioids (level 3). Exclusion criteria are: no inaugural ACS, homeless patients, pregnancy, deprived of their liberty or under guardianship, unable to understand the purpose and conditions of carrying out the study, unable to give consent. Results : From march 2016 to june 2017, 112 adult patients were included in Africa and 122 adults in Europe. Patients in Africa were younger than in Europe with a mean age, respectively, of 26 ± 7 yrs and 29 ± 8 yrs (p=0.008). Sex ratio (M/F) in Africa was 0.84 and 0.95 in Europe. Use of hydroxyurea was significantly higher in Europe (65%) than in Africa (6%) (p Discussion and conclusion: Our preliminary results provide epidemiologic insights about VOC and ACS incidence in Europe and Africa. A better availability of HU treatment in Africa is a health care priority. Disclosures Galacteros: Addmedica: Membership on an entity9s Board of Directors or advisory committees. Bartolucci: GBT: Membership on an entity9s Board of Directors or advisory committees; Fondation Fabre: Research Funding; Addmedica: Research Funding; Novartis US: Membership on an entity9s Board of Directors or advisory committees.

Journal of Clinical Medicine, 2022

Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (C... more Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (CVADs) are commonly used for chronic transfusions and iron chelation in this population. CVADs are an additional venous thromboembolism (VTE) risk factor. The role of thromboprophylaxis in this setting is uncertain. The objectives are: (1) to determine whether thromboprophylaxis reduces VTE risk in SCD patients with CVAD and (2) to explore characteristics associated with VTE risk. We identified adults with SCD and CVAD intended for chronic use (≥3 months) at two comprehensive SCD centers. Thromboprophylaxis presence; type; intensity; and patient-, catheter-, and treatment-related VTE risk factors were recorded. Among 949 patients, 49 had a CVAD (25 without and 24 with VTE prophylaxis). Thromboprophylaxis type and intensity varied widely. Patients without thromboprophylaxis had higher VTE rates (rate ratio (RR) = 4.0 (95% confidence interval: 1.2–12.6), p = 0.02). Hydroxyurea was associate...

American Journal of Hematology, 2021

State Cancer Institute, Indira Gandhi Institute of Medical Sciences, Patna, India Cross Cancer In... more State Cancer Institute, Indira Gandhi Institute of Medical Sciences, Patna, India Cross Cancer Institute, Edmonton, Alberta, Canada Kings College Hospital NHS Foundation Trust, London, UK University Hospitals of Leicester NHS Trust, Leicester, UK City Clinical Hospital #40, Moscow, Russian Federation Baylor University Medical Center, Dallas, Texas, USA University of Nantes, Nantes, France Karyopharm Therapeutics, Newton, Massachusetts, USA Dana-Farber Cancer Institute, Boston, Massachusetts, USA Medical University of Silesia, Katowice, Poland

Blood, 2021

Allogeneic bone marrow transplantation (BMT) is the only curative treatment available for sickle ... more Allogeneic bone marrow transplantation (BMT) is the only curative treatment available for sickle cell disease (SCD). The effectiveness of the treatment is primarily assessed by the chimerism rate, which is the fraction of cells derived from the donor's hematopoietic stem cells (HSCs) relative to the recipient's cells. New less toxic conditioning agents, described as reduced, nonmyeloablative (as opposed to myeloablative conditioning agents) are now used for BMT with sibling donors. Moreover, most sibling donors are sickle cell trait carriers (heterozygous A/S genotype). In the absence of a test performed on erythroid cells, the rate of chimerism is currently assessed either on different subpopulations of myeloid cells by molecular biology techniques or on hemoglobin measurement by chromatography (HPLC). However, the percentage of myeloid chimerism is rarely representative of the percentage of erythroid chimerism in these patients and HPLC cannot allow analysis at the single ...

Blood, 2021

Introduction Sickle cell disease is a genetic disease with acute and chronic complications. Pedia... more Introduction Sickle cell disease is a genetic disease with acute and chronic complications. Pediatric mortality has decreased in recent decades with the introduction of systematic antibiotic therapy, preventive management of cerebral vasculopathy and therapeutic education of families. However, in the absence of cohort follow-up at birth, life expectancy, which is a different concept from age at death, cannot be assessed. In this retrospective, monocentric study, we describe causes and circumstances of death, acute chronic complications, long-term treatments and baseline biology of these patients. It seems important to analyze the risks of morbidity and mortality in order to decide on the necessary preventive measures. Material and method: Records of patients deceased between 2000 and 2020, from the national referral center (Henri Mondor Hospital), were retrospectively reviewed. The referral center follows 3500 patients. All deaths reported to the hospital, by families, other hospita...

Blood, 2021

INTRODUCTION Vaso-occlusive crisis (VOC), hallmark of sickle-cell disease (SCD), is the first cau... more INTRODUCTION Vaso-occlusive crisis (VOC), hallmark of sickle-cell disease (SCD), is the first cause of patients' emergency room (ER) admissions and hospitalizations. Acute chest syndrome (ACS) is a life-threatening complication that can occur during VOC and prolong hospitalization and is one of the main causes of death in SCD patients. The PRESEV score, established by team members and colleagues, assesses the risk of developing ACS (Bartolucci et al., 2016). In addition, the score has been validated by an international multicenter study, involving 13 centers, distributed in five different countries (PRESEV 2 - ASH 2020). Throughout the first wave of the Covid-19 pandemic, VOC management for SCD patients was a major concern. Our sickle cell referral center set up a hotline to monitor patients suffering from VOC daily, and organized the deployment of home-care services when required. The success of this system during the first wave of the pandemic led to the establishment of DREPA...

La Presse Médicale Formation, 2021

Blood, 2020

INTRODUCTION Besides many problematics the Covid-19 pandemic has triggered, one issue remains the... more INTRODUCTION Besides many problematics the Covid-19 pandemic has triggered, one issue remains the care of chronically ill patients. Regarding sickle cell disease (SCD), patients often present co-morbidities that could predispose them to poor outcome if they get infected. Vaso-occlusive crisis (VOC), a characteristic manifestation of SCD, is the first cause of patients 'hospitalizations. Here, we describe how our sickle cell referral center has managed outpatient care, with the constant preoccupation of minimizing risks for our patients and avoid them unnecessary trips to heavily burdened hospital settings. DESCRIPTION OF SETTING With the outbreak of Covid-19, our primary obsession was to provide continuous care for our patients, while ensuring their safety. All appointments were canceled when possible and patients were instructed to comply with the national lock down procedures. A hotline and helpdesk were setup as the first stage of our structure. When patients described sympto...

Blood

Sudden death is 1 of the leading causes of death in adults with sickle cell anemia (SCA) but its ... more Sudden death is 1 of the leading causes of death in adults with sickle cell anemia (SCA) but its etiology remains mostly unknown. Ventricular arrhythmia (VA) carries an increased risk of sudden death; however, its prevalence and determinants in SCA are poorly studied. This study aimed to identify the prevalence and predictors of VA in patients with SCA. From 2019 to 2022, 100 patients with SCA were referred to the physiology department to specifically analyze cardiac function and prospectively included in the DREPACOEUR registry. They underwent a 24-hour electrocardiogram monitoring (24h-Holter), transthoracic echocardiography, and laboratory tests on the same day. The primary end point was the occurrence of VA, defined as sustained or nonsustained ventricular tachycardia (VT), >500 premature ventricular contractions (PVCs) on 24h-Holter, or a recent history of VT ablation. The mean patient age was 46 ± 13 years, and 48% of the patients were male. Overall, VA was observed in 22 (...

Transfusion Clinique et Biologique

Introduction: Because of iron overload complications, thrombosis and infectious predisposition, p... more Introduction: Because of iron overload complications, thrombosis and infectious predisposition, patients with severe forms of thalassemia are likely to be at increased risk of COVID-19 complications. Results: A national survey conducted during the year 2020 across the French reference centers for hemoglobinopathies identified 16 cases of COVID-19 confirmed by RT-PCR in beta-thalassemia patients. Their age ranged from 11 months to 60 years. 15 patients were transfusion dependent and 6 were splenectomized. Concerning iron-overload related complications, none had diabetes or cirrhosis and only one had experienced heart failure. All 4 pediatric patients were pauci-symptomatic during the viral episode. Three patients (41, 49 and 57 years old) developed COVID-19 pneumonia requiring oxygen-therapy without the need for mechanical ventilation. Neutropenia (absolute neutrophils count <0.5 10 9/L) was observed in 2 patients receiving long-term treatment with hydroxycarbamide and deferiprone. No thrombosis event, organ failure or death occurred. All patients recovered. Conclusion: Severity of COVID-19 in this population of early-aged patients appeared increased compared to the general population but remained mild to moderate as already reported in the few series reported in the literature. Occurrence of adverse events related to chronic treatment administered in thalassemia disease may be favored by the infectious episode.

Transfusion Clinique et Biologique, 2021

Frontiers in Neuroscience, 2016

Neurodegenerative diseases affect a significant portion of the aging population. Several lines of... more Neurodegenerative diseases affect a significant portion of the aging population. Several lines of evidence suggest a positive association between environmental exposures, which are common and cumulative in a lifetime, and development of neurodegenerative diseases. Environmental or occupational exposure to manganese (Mn) has been implicated in neurodegeneration due to its ability to induce mitochondrial dysfunction, oxidative stress, and α-synuclein (α-Syn) aggregation. The role of the α-Syn protein visa-vis Mn is controversial, as it seemingly plays a duplicitous role in neuroprotection and neurodegeneration. α-Syn has low affinity for Mn, however an indirect interaction cannot be ruled out. In this review we will examine the current knowledge surrounding the interaction of α-Syn and Mn in neurodegenerative process.

Objetivos: Analizar una serie de pacientes diagnosticados de enfermedad de Kikuchi (EK, linfadeni... more Objetivos: Analizar una serie de pacientes diagnosticados de enfermedad de Kikuchi (EK, linfadenitis histiocítica necrotizante) en nuestro hospital, y describir la asociación con enfermedades autoinmunes sistémicas (EAS) y onco-hematológicas. Métodos: Estudio descriptivo de las características clínicas y epidemiológicos de 18 pacientes con EK, con diagnóstico anatomopatológico. Se revisaron las historias clínicas retrospectivamente desde el 1993 hasta el 2014. Resultados: Durante el estudio, se obtuvo el diagnóstico EK con anatomía patológica de linfadenitis histiocítica necrotizante en 18 pacientes. De ellos, 16/18 (88%) eran caucásicos, 1/18 (6%) africano y 1/18 (6%) asiático. Predominó el sexo femenino con 16/17 (94%). La edad media al diagnóstico fue de 30 años (± 11,64). Clínicamente, 12/18 (65%) presentaron fiebre y todos debutaron con enfermedad ganglionar cervical. Dos pacientes (11%) presentaron adenopatías inguinales. Síntomas como rash o artralgias estuvieron presentes en...

Enfermedades Infecciosas y Microbiología Clínica, 2011

cervantesvirtual.com

La Biblioteca Virtual Miguel de Cervantes, la primera en lengua castellana, es un fondo bibliográ... more La Biblioteca Virtual Miguel de Cervantes, la primera en lengua castellana, es un fondo bibliográfico con obras de Literatura, Historia, Ciencias, etc., de libre acceso. Incluye trabajos de investigación, catálogo en otras lenguas y bibliotecas del mundo.

American Journal of Hematology

American Journal of Hematology

Blood, Dec 7, 2017

Introduction : Vaso-Occlusive Crisis (VOC), the most common manifestation of sickle cell disease ... more Introduction : Vaso-Occlusive Crisis (VOC), the most common manifestation of sickle cell disease (SCD), is the first cause of death, particularly when complicated by an acute chest syndrome (ACS). In the PRESEV1 study, we developed a predictive score of secondary ACS in SCD patient admitted in the hospital for VOC (AUC = 0.840 [95% CI: 0.780 - 0.900]), with a negative predictive value of 98.8% for the low level risk and a positive predictive value of 39.5%. The aim of the PRESEV-2 study is to validate the predictive score of ACS on adults and children with SCD in Europe and Africa. Herein we present preliminary data on 235 adults with a severe VOC included in 11 referral centers from 4 countries. Patients and Methods: We conduct an international multicenter prospective observational study. The primary outcome measure is the occurrence of an ACS. The ACS is defined as a new auscultatory abnormality (crepitation or bronchial breathing) OR the association of a new radiologic infiltrate AND chest pain or decreased breath sounds. Secondary outcome measures are length of hospitalization, transfusion, morphine consumption, mortality and hospitalization in intensive care unit. Inclusion criteria are: SS or S-b0 thalassemia patients, VOC admitted at the emergency unit (VOC is defined as pain or tenderness affecting at least one part of the body not attributable to other causes) that required hospitalization and opioids (level 3). Exclusion criteria are: no inaugural ACS, homeless patients, pregnancy, deprived of their liberty or under guardianship, unable to understand the purpose and conditions of carrying out the study, unable to give consent. Results : From march 2016 to june 2017, 112 adult patients were included in Africa and 122 adults in Europe. Patients in Africa were younger than in Europe with a mean age, respectively, of 26 ± 7 yrs and 29 ± 8 yrs (p=0.008). Sex ratio (M/F) in Africa was 0.84 and 0.95 in Europe. Use of hydroxyurea was significantly higher in Europe (65%) than in Africa (6%) (p Discussion and conclusion: Our preliminary results provide epidemiologic insights about VOC and ACS incidence in Europe and Africa. A better availability of HU treatment in Africa is a health care priority. Disclosures Galacteros: Addmedica: Membership on an entity9s Board of Directors or advisory committees. Bartolucci: GBT: Membership on an entity9s Board of Directors or advisory committees; Fondation Fabre: Research Funding; Addmedica: Research Funding; Novartis US: Membership on an entity9s Board of Directors or advisory committees.

Journal of Clinical Medicine, 2022

Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (C... more Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (CVADs) are commonly used for chronic transfusions and iron chelation in this population. CVADs are an additional venous thromboembolism (VTE) risk factor. The role of thromboprophylaxis in this setting is uncertain. The objectives are: (1) to determine whether thromboprophylaxis reduces VTE risk in SCD patients with CVAD and (2) to explore characteristics associated with VTE risk. We identified adults with SCD and CVAD intended for chronic use (≥3 months) at two comprehensive SCD centers. Thromboprophylaxis presence; type; intensity; and patient-, catheter-, and treatment-related VTE risk factors were recorded. Among 949 patients, 49 had a CVAD (25 without and 24 with VTE prophylaxis). Thromboprophylaxis type and intensity varied widely. Patients without thromboprophylaxis had higher VTE rates (rate ratio (RR) = 4.0 (95% confidence interval: 1.2–12.6), p = 0.02). Hydroxyurea was associate...

American Journal of Hematology, 2021

State Cancer Institute, Indira Gandhi Institute of Medical Sciences, Patna, India Cross Cancer In... more State Cancer Institute, Indira Gandhi Institute of Medical Sciences, Patna, India Cross Cancer Institute, Edmonton, Alberta, Canada Kings College Hospital NHS Foundation Trust, London, UK University Hospitals of Leicester NHS Trust, Leicester, UK City Clinical Hospital #40, Moscow, Russian Federation Baylor University Medical Center, Dallas, Texas, USA University of Nantes, Nantes, France Karyopharm Therapeutics, Newton, Massachusetts, USA Dana-Farber Cancer Institute, Boston, Massachusetts, USA Medical University of Silesia, Katowice, Poland

Blood, 2021

Allogeneic bone marrow transplantation (BMT) is the only curative treatment available for sickle ... more Allogeneic bone marrow transplantation (BMT) is the only curative treatment available for sickle cell disease (SCD). The effectiveness of the treatment is primarily assessed by the chimerism rate, which is the fraction of cells derived from the donor's hematopoietic stem cells (HSCs) relative to the recipient's cells. New less toxic conditioning agents, described as reduced, nonmyeloablative (as opposed to myeloablative conditioning agents) are now used for BMT with sibling donors. Moreover, most sibling donors are sickle cell trait carriers (heterozygous A/S genotype). In the absence of a test performed on erythroid cells, the rate of chimerism is currently assessed either on different subpopulations of myeloid cells by molecular biology techniques or on hemoglobin measurement by chromatography (HPLC). However, the percentage of myeloid chimerism is rarely representative of the percentage of erythroid chimerism in these patients and HPLC cannot allow analysis at the single ...

Blood, 2021

Introduction Sickle cell disease is a genetic disease with acute and chronic complications. Pedia... more Introduction Sickle cell disease is a genetic disease with acute and chronic complications. Pediatric mortality has decreased in recent decades with the introduction of systematic antibiotic therapy, preventive management of cerebral vasculopathy and therapeutic education of families. However, in the absence of cohort follow-up at birth, life expectancy, which is a different concept from age at death, cannot be assessed. In this retrospective, monocentric study, we describe causes and circumstances of death, acute chronic complications, long-term treatments and baseline biology of these patients. It seems important to analyze the risks of morbidity and mortality in order to decide on the necessary preventive measures. Material and method: Records of patients deceased between 2000 and 2020, from the national referral center (Henri Mondor Hospital), were retrospectively reviewed. The referral center follows 3500 patients. All deaths reported to the hospital, by families, other hospita...

Blood, 2021

INTRODUCTION Vaso-occlusive crisis (VOC), hallmark of sickle-cell disease (SCD), is the first cau... more INTRODUCTION Vaso-occlusive crisis (VOC), hallmark of sickle-cell disease (SCD), is the first cause of patients' emergency room (ER) admissions and hospitalizations. Acute chest syndrome (ACS) is a life-threatening complication that can occur during VOC and prolong hospitalization and is one of the main causes of death in SCD patients. The PRESEV score, established by team members and colleagues, assesses the risk of developing ACS (Bartolucci et al., 2016). In addition, the score has been validated by an international multicenter study, involving 13 centers, distributed in five different countries (PRESEV 2 - ASH 2020). Throughout the first wave of the Covid-19 pandemic, VOC management for SCD patients was a major concern. Our sickle cell referral center set up a hotline to monitor patients suffering from VOC daily, and organized the deployment of home-care services when required. The success of this system during the first wave of the pandemic led to the establishment of DREPA...

La Presse Médicale Formation, 2021

Blood, 2020

INTRODUCTION Besides many problematics the Covid-19 pandemic has triggered, one issue remains the... more INTRODUCTION Besides many problematics the Covid-19 pandemic has triggered, one issue remains the care of chronically ill patients. Regarding sickle cell disease (SCD), patients often present co-morbidities that could predispose them to poor outcome if they get infected. Vaso-occlusive crisis (VOC), a characteristic manifestation of SCD, is the first cause of patients 'hospitalizations. Here, we describe how our sickle cell referral center has managed outpatient care, with the constant preoccupation of minimizing risks for our patients and avoid them unnecessary trips to heavily burdened hospital settings. DESCRIPTION OF SETTING With the outbreak of Covid-19, our primary obsession was to provide continuous care for our patients, while ensuring their safety. All appointments were canceled when possible and patients were instructed to comply with the national lock down procedures. A hotline and helpdesk were setup as the first stage of our structure. When patients described sympto...