THROMBIN ACTIVATABLE FIBRINOLYSIS INHIBITOR (TAFI): Relationship to Hemostatic Alteration in Patients with β-Thalassemia

Mokhtar, Galila M.; Matter, Randa M.; Shawki, Hadeel Ahmed; Aziz, Manal M. Abdel

doi:10.3109/08880011003739448

Cited by 8 publications

(8 citation statements)

References 38 publications

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“…Similar findings were reported in a previous study on thalassemic children. 20 The findings of this study support the suggestion raised in previous studies that splenectomized thalassemic children, even if asymptomatic are actually in a state of low-grade consumptive coagulopathy with continuous thrombin generation and enhanced fibrinolysis, leading to a subclinical process of thrombosis. 17 This was thought to contribute to the high frequency of ischemic brain lesions which was associated with asymptomatic brain damage as detected by magnetic resonance imaging in thalassemic patients.…”

Section: Discussionsupporting

confidence: 90%

Coagulation defects in thalassemic patients

Abosdera

Almasry

Abdel-Moneim

2017

Pediatrics & Neonatology

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Section: Discussionsupporting

confidence: 90%

Coagulation defects in thalassemic patients

Abosdera

Almasry

Abdel-Moneim

2017

Pediatrics & Neonatology

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“…11,12 Epistaxis was reported in 17.5% in Egyptian β-thalassemia patients. 13 Mucosal and subcutaneous bleedings are the typical presentation of platelet-type hemorrhagic diathesis. Among our study patients both the bleeding phenotype as well as the hemostatic workup were suggestive of primary hemostatic disorder (platelet-type).…”

Section: Variablesmentioning

confidence: 99%

Navigating Hemostasis of Bleeding Among Children With β-Thalassemia

El‐Beshlawy¹,

Rabah

Hamed³

et al. 2022

Journal of Pediatric Hematology/Oncology

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Bleeding phenotype is reported in β-thalassemia patients. However, the underlying etiology remains elusive. We aimed to assess coagulation profile and the platelet aggregation in β-thalassemia children with bleeding diathesis. Fifty β-thalassemia children with a positive bleeding history were recruited. Bleeding phenotype was explored through full history taking and thorough clinical examination. Complete blood count, prothrombin time, international normalized ratio, and platelets aggregometry were performed for children with negative workup. Mucosal bleeding was manifest among most of our patients (96%). Two-third of patients had decreased aggregation with ristocetin (68%), adenine di-phosphate (64%), and arachidonic acid (64%). While half of the patients (48%) had deficient response to epinephrine. Collagen, ristocetin, and arachidonic acid induced aggregation were negatively correlated to frequency of blood transfusion (P=0.021, r=−0.325; P<0.001, r=−0.465; P=0.018, r=−0.333, respectively). Aggregation to collagen and epinephrine demonstrated a negative correlation with age (P=0.04, r=−0.287; P=0.03, r=−0.315). Deferiprone was associated with a deficient response to ristocetin and collagen when compared with deferasirox or no chelation (P=0.021 and 0.006, respectively). Impaired ristocetin response was linked to hydroxyurea (P=0.035). Platelets function defect should be considered in β-thalassemia patients with bleeding symptoms.

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“…(We note that sometimes fibrinolysis is assessed via D-dimer formation, 163 but that when this is increased one cannot normally tell whether that is due to an increase in fibrinolysis or simply because there had previously been hypercoagulation.) Note that in the case of b-thalassaemia there is a particularly noticeable amount of hypercoagulation [323][324][325][326][327][328][329][330][331][332][333][334][335] but that fibrinolysis seems to be enhanced, 324,336,337 albeit not by enough to overcome the hypercoagulation. The same seems to be true of psoriasis 66,[338][339][340] and of asthma.…”

Section: The Relationship Between Hypercoagulability and Hypofibrinolmentioning

confidence: 99%

The simultaneous occurrence of both hypercoagulability and hypofibrinolysis in blood and serum during systemic inflammation, and the roles of iron and fibrin(ogen)

Kell

Pretorius

2014

Integrative Biology

127

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Although the two phenomena are usually studied separately, we summarise a considerable body of literature to the effect that a great many diseases involve (or are accompanied by) both an increased tendency for blood to clot (hypercoagulability) and the resistance of the clots so formed (hypofibrinolysis) to the typical, 'healthy' or physiological lysis. We concentrate here on the terminal stages of fibrin formation from fibrinogen, as catalysed by thrombin. Hypercoagulability goes hand in hand with inflammation, and is strongly influenced by the fibrinogen concentration (and vice versa); this can be mediated via interleukin-6. Poorly liganded iron is a significant feature of inflammatory diseases, and hypofibrinolysis may change as a result of changes in the structure and morphology of the clot, which may be mimicked in vitro, and may be caused in vivo, by the presence of unliganded iron interacting with fibrin(ogen) during clot formation. Many of these phenomena are probably caused by electrostatic changes in the iron-fibrinogen system, though hydroxyl radical (OH ) formation can also contribute under both acute and (more especially) chronic conditions. Many substances are known to affect the nature of fibrin polymerised from fibrinogen, such that this might be seen as a kind of bellwether for human or plasma health. Overall, our analysis demonstrates the commonalities underpinning a variety of pathologies as seen in both hypercoagulability and hypofibrinolysis, and offers opportunities for both diagnostics and therapies.

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THROMBIN ACTIVATABLE FIBRINOLYSIS INHIBITOR (TAFI): Relationship to Hemostatic Alteration in Patients with β-Thalassemia

Cited by 8 publications

References 38 publications

Coagulation defects in thalassemic patients

Coagulation defects in thalassemic patients

Navigating Hemostasis of Bleeding Among Children With β-Thalassemia

The simultaneous occurrence of both hypercoagulability and hypofibrinolysis in blood and serum during systemic inflammation, and the roles of iron and fibrin(ogen)

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