2018
Mehdid F, Rekkab N, Oukid S, Abad MT, Bradai M, Hamdi S, Boukhemia F, Hamladji RM, Nacer RA, Allouda M, et al. Epidemiological Study of Aplastic Anemia in Algeria for 844 Cases over 10 Years (2007-2016). BloodBlood. 2018;132 :5109.
AbstractIntroduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes. Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level. Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards. Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat Disclosures No relevant conflicts of interest to declare.
Mehdid F, Rekkab N, Oukid S, Abad MT, Bradai M, Hamdi S, Boukhemia F, Hamladji RM, Nacer RA, Allouda M, et al. Epidemiological Study of Aplastic Anemia in Algeria for 844 Cases over 10 Years (2007-2016). BloodBlood. 2018;132 :5109.
AbstractIntroduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes. Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level. Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards. Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat Disclosures No relevant conflicts of interest to declare.
Mehdid F, Rekkab N, Oukid S, Abad MT, Bradai M, Hamdi S, Boukhemia F, Hamladji RM, Nacer RA, Allouda M, et al. Epidemiological Study of Aplastic Anemia in Algeria for 844 Cases over 10 Years (2007-2016). BloodBlood. 2018;132 :5109.
AbstractIntroduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes. Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level. Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards. Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat Disclosures No relevant conflicts of interest to declare.
Mehdid F, Rekkab N, Oukid S, Abad MT, Bradai M, Hamdi S, Boukhemia F, Hamladji RM, Nacer RA, Allouda M, et al. Epidemiological Study of Aplastic Anemia in Algeria for 844 Cases over 10 Years (2007-2016). BloodBlood. 2018;132 :5109.
AbstractIntroduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes. Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level. Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards. Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat Disclosures No relevant conflicts of interest to declare.
Mehdid F, Rekkab N, Oukid S, Abad MT, Bradai M, Hamdi S, Boukhemia F, Hamladji RM, Nacer RA, Allouda M, et al. Epidemiological Study of Aplastic Anemia in Algeria for 844 Cases over 10 Years (2007-2016). BloodBlood. 2018;132 :5109.
AbstractIntroduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes. Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level. Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards. Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat Disclosures No relevant conflicts of interest to declare.
Mehdid F, Rekkab N, Oukid S, Abad MT, Bradai M, Hamdi S, Boukhemia F, Hamladji RM, Nacer RA, Allouda M, et al. Epidemiological Study of Aplastic Anemia in Algeria for 844 Cases over 10 Years (2007-2016). BloodBlood. 2018;132 :5109.
AbstractIntroduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes. Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level. Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards. Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat Disclosures No relevant conflicts of interest to declare.
Mehdid F, Rekkab N, Oukid S, Abad MT, Bradai M, Hamdi S, Boukhemia F, Hamladji RM, Nacer RA, Allouda M, et al. Epidemiological Study of Aplastic Anemia in Algeria for 844 Cases over 10 Years (2007-2016). BloodBlood. 2018;132 :5109.
AbstractIntroduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes. Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level. Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards. Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat Disclosures No relevant conflicts of interest to declare.
Mehdid F, Rekkab N, Oukid S, Abad MT, Bradai M, Hamdi S, Boukhemia F, Hamladji RM, Nacer RA, Allouda M, et al. Epidemiological Study of Aplastic Anemia in Algeria for 844 Cases over 10 Years (2007-2016). BloodBlood. 2018;132 :5109.
AbstractIntroduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes. Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level. Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards. Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.
Grifi F, Djenouni A, Bougherira S, Abad MT, Boucherit C, Boudjerra N, Zidani N, Aboura C, Aribi A, Belhani M, et al. Epidemiological Study on β-Thalassemia in Algeria. BloodBlood. 2018;132 :4902.
AbstractIntroduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.