Thrombotic Events and Anticoagulants in Beta-thalassemia Patients with Focus on Anticoagulants for Atrial Fibrillation: A Brief Review

Bahrani, Saeide; Teimouri-jervekani, Zahra; Sadeghi, Masoumeh

doi:10.1016/j.cpcardiol.2021.100912

Cited by 5 publications

(2 citation statements)

References 41 publications

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“…Initially, the gold standard therapy was considered the administration of vitamin K antagonists, which in turn required frequent blood samples to monitor international normalized ratio (INR) and thus more venous accesses. Recently, the use of direct oral anticoagulants (DOACs), which exert their anticoagulant action by inhibiting a specific factor of the coagulation cascade is preferred, inducing, compared with anti-vitamin K drugs, a more stable anticoagulation action without the need for continuous INR monitoring and consequent reduction of venous accesses [ 12 , 22 ].…”

Section: Discussionmentioning

confidence: 99%

Characterization and Clinical Assessment of a Peculiar Case of Hemolytic Anemia

Castelgrande,

Viola,

Calabrese

et al. 2024

J Hematol

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Thalassemic diseases are characterized by a reduced (β + ) or absent (β 0 ) synthesis of the globin chains of hemoglobin (Hb) due to genetic mutations. β-thalassemia was more frequent in the Mediterranean area, but now it is diffused worldwide. Three possible genetic forms can be distinguished: β 0 /β 0 , the most severe (Cooley’s disease); β 0 /β + of intermediate severity; β + /β + associated with β-thalassemia intermedia or minor. Recently, a clinical non-genetic classification has been proposed: transfusion-dependent thalassemia (TDT), requiring regular lifetime blood transfusions, and non-transfusion-dependent thalassemia (NTDT), requiring occasional transfusions to manage acute cases. In this report, we studied a patient whose blood count indicated a severe anemia but also showed thrombocytosis, leukocytosis, and an elevated number of nucleated red blood cells (NRBC). These altered blood parameters suggested initially a possible diagnosis of hemoglobinopathy or myeloproliferative syndrome. The molecular and genetic analyses demonstrated the presence of HbF (5.3%) and HbA2 (7.7%) and the presence of the homozygote mutation (IVS1.6T>C) in the β-globin gene. According to these data, a diagnosis of β-thalassemia intermedia form has been proposed. Nevertheless, the clinical condition, the presence of thrombocytosis, leukocytosis, an elevated number of NRBC, and the frequent blood transfusions lead to reclassification of the patient as TDT subject. Consequently, this result suggests that a unique genotype-phenotype correlation is not possible in the presence of β + mutations since other concomitant pathologies can exacerbate the disease.

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Section: Discussionmentioning

confidence: 99%

Characterization and Clinical Assessment of a Peculiar Case of Hemolytic Anemia

Castelgrande,

Viola,

Calabrese

et al. 2024

J Hematol

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“…Despite the worldwide adoption of NOACs in the general population, very little is reported in the literature regarding their use in patients with β-thalassemia [ 20 ]. The use of rivaroxaban only has been described in small case series and case reports [ 21 , 22 ].…”

Section: Introductionmentioning

confidence: 99%

Non-Vitamin K Antagonist Oral Anticoagulants in Patients with β-Thalassemia

Malagù

Longo

Marchini

et al. 2023

Biology

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Background. Patients with β-thalassemia have a high incidence of atrial fibrillation (AF) and other supraventricular arrhythmias. The use of non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prophylaxis in patients with β-thalassemia has not been systematically evaluated. Methods. We enrolled patients with transfusion-dependent β-thalassemia, who were on treatment with NOACs for thromboembolic prophylaxis of supraventricular arrhythmias. Data on thromboembolic and bleeding events were collected. Results. Eighteen patients were enrolled. The patients had a history of AF (sixteen), typical atrial flutter (five), and atypical atrial flutter (four). The patients were treated with dabigatran (seven), apixaban (five), rivaroxaban (four) or edoxaban (two). The mean follow-up duration was 22 ± 15 months. No thromboembolic events were reported. No major bleedings were observed. Three patients had non-major bleeding events. Two patients reported dyspepsia during treatment with dabigatran and were shifted to a different NOAC. Conclusions. Our study suggests the efficacy and safety of NOACs in patients affected by transfusion-dependent β-thalassemia.

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Hypercoagulability in hemoglobinopathies: Decoding the thrombotic threat

Bou‐Fakhredin,

Cappellini,

Taher

et al. 2024

American J Hematol

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Beta (β)‐thalassemia and sickle cell disease (SCD) are characterized by a hypercoagulable state, which can significantly influence organ complication and disease severity. While red blood cells (RBCs) and erythroblasts continue to play a central role in the pathogenesis of thrombosis in β‐thalassemia and SCD, additional factors such as free heme, inflammatory vasculopathy, splenectomy, among other factors further contribute to the complexity of thrombotic risk. Thus, understanding the role of the numerous factors driving this hypercoagulable state will enable healthcare practitioners to enhance preventive and treatment strategies and develop novel therapies for the future. We herein describe the pathogenesis of thrombosis in patients with β‐thalassemia and SCD. We also identify common mechanisms underlying the procoagulant profile of hemoglobinopathies translating into thrombotic events. Finally, we review the currently available prevention and clinical management of thrombosis in these patient populations.

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Thrombotic Events and Anticoagulants in Beta-thalassemia Patients with Focus on Anticoagulants for Atrial Fibrillation: A Brief Review

Cited by 5 publications

References 41 publications

Characterization and Clinical Assessment of a Peculiar Case of Hemolytic Anemia

Characterization and Clinical Assessment of a Peculiar Case of Hemolytic Anemia

Non-Vitamin K Antagonist Oral Anticoagulants in Patients with β-Thalassemia

Hypercoagulability in hemoglobinopathies: Decoding the thrombotic threat

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