Hypercoagulability in β-thalassemia: a status quo

Cappellini, Maria Domenica; Poggiali, Erika; Taher, Ali T.; Musallam, Khaled M.

doi:10.1586/ehm.12.42

Cited by 77 publications

(68 citation statements)

References 55 publications

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“…The contribution of hypercoagulability to the mortality and morbidity of thalassemia patients has long been understood [44]. Laboratory studies have elucidated the many factors contributing to hypercoagulability of this disease [45]. Of concern, hypercoagulability can contribute to cerebral ischemia which may initially be clinically silent [46].…”

Section: Hypercoagulabilitymentioning

confidence: 99%

“…Of concern, hypercoagulability can contribute to cerebral ischemia which may initially be clinically silent [46]. Nonetheless despite the importance of these findings, there are still no clearcut guidelines on prevention of these complications [45], despite the availability of many new antithrombotic agents. Clearly this is an area in which further clinical research is urgently needed.…”

Section: Hypercoagulabilitymentioning

confidence: 99%

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Thalassemia 2016: Modern medicine battles an ancient disease

Rund

2015

American J Hematol

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Thalassemia was first clinically described nearly a century ago and treatment of this widespread genetic disease has greatly advanced during this period. DNA‐based diagnosis elucidated the molecular basis of the disease and clarified the variable clinical picture. It also paved the way for modern methods of carrier identification and prevention via DNA‐based prenatal diagnosis. Every aspect of supportive care, including safer blood supply, more regular transfusions, specific monitoring of iron overload, parenteral and oral chelation, and other therapies, has prolonged life and improved the quality of life of these patients. Significant advances have also been made in allogenic bone marrow transplantation, the only curative therapy. Recently, there has been a rejuvenated interest in studying thalassemia at the basic science level, leading to the discovery of previously unknown mechanisms leading to anemia and enabling the development of novel therapies. These will potentially improve the treatment of, and possibly cure the disease. Pathways involving activin receptors, heat shock proteins, JAK2 inhibitors and macrophage targeted therapy, among others, are being studied or are currently in clinical trials for treating thalassemia. Novel types of genetic therapies are in use or under investigation. In addition to the challenges of treating each individual patient, the longer survival of thalassemia patients has raised considerations regarding worldwide control of thalassemia, since prevention is not universally implemented. This review will trace a number of the original medical milestones of thalassemia diagnosis and treatment, as well as some of the most recent developments which may lead to innovative therapeutic modalities. Am. J. Hematol. 91:15–21, 2016. © 2015 Wiley Periodicals, Inc.

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Section: Hypercoagulabilitymentioning

confidence: 99%

Section: Hypercoagulabilitymentioning

confidence: 99%

Thalassemia 2016: Modern medicine battles an ancient disease

Rund

2015

American J Hematol

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“…9 In addition, they are more prone to thrombotic-related complications than patients affected by b-thalassemia major. 10,11 Furthermore, the phenotype of these patients might also change over time, as NTDT patients often become transfusion dependent.…”

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confidence: 99%

-thalassemias: paradigmatic diseases for scientific discoveries and development of innovative therapies

2015

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b-thalassemias are monogenic disorders characterized by defective synthesis of the b-globin chain, one of the major components of adult hemoglobin. A large number of mutations in the b-globin gene or its regulatory elements have been associated with b-thalassemias. Due to the complexity of the regulation of the b-globin gene and the role of red cells in many physiological processes, patients can manifest a large spectrum of phenotypes, and clinical requirements vary from patient to patient. It is important to consider the major differences in the light of potential novel therapeutics. This review summarizes the main discoveries and mechanisms associated with the synthesis of b-globin and abnormal erythropoiesis, as well as current and novel therapies. ABSTRACTThe complex phenotype of b-thalassemias b-thalassemias are monogenic disorders characterized by reduced or no synthesis of the b-globin chain, one of the major components of adult hemoglobin (HbA). Several hundred mutations in the b-globin gene or regulatory elements have been associated with b-thalassemias.

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“…Hal tersebut sejalan dengan penelitian Canatan dan Zorlu 29 serta Abbas 30 bahwa peningkatan jumlah trombosit meningkatkan pembentukan trombin dan stimulasi aktivitas prokoagulan. 31 Abnormalitas eritrosit pada thalassemia menyebabkan fosfatidilserin berada di sisi luar membran eritrosit dan menyebabkan peningkatan aktivitas dan adhesi trombosit sehingga risiko hiperkoagulasi meningkat. Keterangan: *total 80 subjek, 7 subjek tanpa terapi kelasi besi…”

Section: Pembahasanunclassified

“…32 Perlu analisis lebih lanjut apakah terdapat gejala klinis, meskipun ringan, terkait hiperkoagulasi seperti keluhan nyeri kepala, pegalpegal, dan parestesi pada ekstremitas. 31,33 Faktor usia bukan merupakan faktor risiko hiperkoagulasi. Penelitian sebelumnya menyatakan bahwa usia anak dini pun telah terjadi hiperkoagulasi, [1][2][3] tetapi tidak menggunakan analisis multivariat.…”

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