Guidelines on Beta-thalassemia major – regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira – 2016

Langhi, Dante Mário; Ubiali, Eugênia Maria Amorim; Marques, José Francisco Comenalli; Veríssimo, Mônica Pinheiro de Almeida; Loggetto, Sandra Regina; Silvinato, Antônio; Bernardo, Wanderley Marques

doi:10.1016/j.bjhh.2016.09.003

Cited by 34 publications

(31 citation statements)

References 15 publications

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“…This indicate that our patients need to critical monitoring and cost, also they are high risk for other infection which are transmit to them during medical care. Same result shows in the previous study [12].…”

Section: Blood Transfusionsupporting

confidence: 87%

Epidemiological Characteristics and Family Relatives among Thalassemic Patients in Sulaimani City, Kurdistan Region, Iraq

Rashid¹

2018

KJAR

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Thalassemia is a well-known inherited hematologic disorder caused by a decrease or an absence of globin production. Patients with thalassemia suffer from chronic hemolytic anemia and its sequelae. A descriptive study was undertaken in Sulaimani Thalassemia and Congenital Blood Disease Center. The self-administered questionnaire was used to 140 patients who had thalassemia and visited to the hospital during the study periods to assess the patients’ characteristics and parent’s relative’s relation to thalassemic patients. Data were entered into spreadsheets and analyzed in Statistical Package for Social Sciences (SPSS) version 22. Descriptive analysis was performed and frequencies and percentages were reported. Associations between categorical variables were checked using Chi-squared test. Out of 140 patients 50.0% of patients were males and 50.0% were female; the mean age was 12.8 years-old. The most frequent thalassemic patents were come from rural area 95.0%. Majority of patients have major type of thalassemia 84.3%. Most of the patients cannot continue to the study accounted 66.4%. About number of children most of the patient’s family had more than 3 children 99.3%. Majority of patient’s parents is carrier for thalassemia and accounted with 90 (65.0%), from those patients with major thalassemia are 77 (85.6) and few cases with minor thalassemia 4 (4.4%). These relations are statistically significant at (p<0.05). Majority of patients who have major type of thalassemia were from rural area followed by urban area 113 (95.8%) vs. 7 (5.0%), these differences are statistically significant at (p≤0.01), from major types of thalassemic patients; 85 (94.4%) of them were both father and mother are carrier of thalassemia. Statistically significant relation was found at (p<0.05). In conclusion, thalassemia is an important cause of morbidity among thalassemic patients. In our community, thalassemia is more frequent in rural population. Therefore, it is a crucial for monitoring and examination of those who are carriers. Moreover, the lack of health education on thalassemia was the major problems related with awareness of families.

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Section: Blood Transfusionsupporting

confidence: 87%

Epidemiological Characteristics and Family Relatives among Thalassemic Patients in Sulaimani City, Kurdistan Region, Iraq

Rashid¹

2018

KJAR

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“…The most commonly used methods are reverse dot blot analysis and primer-specific amplification, with a set of probes or primers complementary to the most common mutations in the population from which the affected individual originated. If targeted mutation analysis fails to detect the mutation, HBB sequence analysis can be used to detect mutations in HBB gene [16].…”

Section: Molecular Genetic Analysismentioning

confidence: 99%

“…The objectives of transfusion therapy is to correct anemia, suppress erythropoiesis and inhibit gastrointestinal iron absorption that occurs in untransfused patients as a result of increased, albeit ineffective, erythropoiesis. The decision to start transfusion therapy in patients with confirmed diagnosis of beta-thalassemia should be based on the presence of severe anemia with hemoglobin (Hb) levels < 7 g/dL and any following clinical criteria: Facial changes, poor growth, spontaneous fractures and clinically significant extramedullary hematopoiesis [16]. The recommended treatment for betathalassemia major involves regular red blood cell transfusions throughout life, usually administered every two to five weeks depending on the transfusion needs of each individual in order to maintain the pre-transfusion level of hemoglobin between 9 and 10.5 g/dL [3] or higher (11-12 g/dL) for patients with cardiac complications and keep post-transfusion hemoglobin levels below 15 g/dL.…”

Section: Transfusion Therapymentioning

confidence: 99%

“…Patients starting regular transfusion regimens should be vaccinated against hepatitis A and B (depending on the age) [30] and their cytomegalovirus status should be assessed. The development of one or more specific anti-red blood cell antibodies (alloimmunization) is an important complication of regular transfusion therapy [16,32]. Before starting transfusion therapy, the patient's blood type must be determined as well as red blood cell phenotyping for at least the C, c, D, E, e and Kell antigens in order to help identify and characterize antibodies in the case of subsequent immunization.…”

Section: Transfusion Therapymentioning

confidence: 99%

“…Before starting transfusion therapy, the patient's blood type must be determined as well as red blood cell phenotyping for at least the C, c, D, E, e and Kell antigens in order to help identify and characterize antibodies in the case of subsequent immunization. Preferably, extended phenotyping should be made including antigens of other blood systems such as Duffy and Kidd and genotyping [16] as well as The use of extended antigen-matched donor blood reduces alloimmunization rates [30]. The risk of transfusion-transmitted infections in thalassemia patients has been greatly reduced since screening for human immunodeficiency virus infections began in 1985 and for hepatitis C in 1991 [33].…”

Section: Transfusion Therapymentioning

confidence: 99%

See 2 more Smart Citations

Beta Thalassemia Major: Overview of Molecular Etiology, Pathophysiology, Current and Novel Therapeutic Approaches

Kababi

Khalfi

Maani

et al. 2020

IBRR

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Major beta thalassemia is a severe form of thalassemia caused by the alteration of two beta globin genes resulting in a defective synthesis of hemoglobin. It is characterized by chronic severe anemia, ineffective erythropoiesis (IE) and iron overload. However although the thransfusion and chelation assosciated constitute the basis of the traitement curently recommended, they do not allow always to control the iron overload induced by pathology and repeated transfusions. Hematopoietic stem cell transplantation (HSCT) has proven to be a definitive treatment for beta thalassemia. However, this procedure is confronted to immunological complications and the small nomber of histocompatible donors. In the face of these therapeutic blocks, much research has been undertaken in recent years leading to the development of a number of promising therapeutic strategies in order to reduce the constraints linked to current chronic treatments, and to move towards an access to healing for all patients. Among other three approaches are envisaged and are in the experimental phase: Gene therapy to restore globin chain imbalance, Improve ineffective erythropoiesis and Improve iron dysregulation. In this article we give a view on the pathophysiology, clinical manifestations, genetic origin of beta-thalassaemia major. The second part presents the therapeutic arsenal currently used, and its limits leading to therapeutic impasse. The last part explores the scientific tracks that present a real therapeutic potential in β-Thalassemia.

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Bone mineral density among Palestinian patients suffering from hemoglobinopathy disorders

Kharroubi

Karmi²,

Shamasneh³

et al. 2020

Arch Osteoporos

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Guidelines on Beta-thalassemia major – regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira – 2016

Cited by 34 publications

References 15 publications

Epidemiological Characteristics and Family Relatives among Thalassemic Patients in Sulaimani City, Kurdistan Region, Iraq

Epidemiological Characteristics and Family Relatives among Thalassemic Patients in Sulaimani City, Kurdistan Region, Iraq

Beta Thalassemia Major: Overview of Molecular Etiology, Pathophysiology, Current and Novel Therapeutic Approaches

Bone mineral density among Palestinian patients suffering from hemoglobinopathy disorders

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