Abnormal Platelet Functions in β Thalassaemia

Eldor, Amiram

doi:10.1111/j.1600-0609.1978.tb02481.x

Cited by 34 publications

(11 citation statements)

References 11 publications

(5 reference statements)

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“…As platelets play a key role in homeostasis and thrombosis, many studies examined whether platelet function is altered in these diseases. Chronic platelet activation in thalassemia was demonstrated by the presence of an increased fraction of platelets carrying the activation markers CD62P (P-selectin) and CD63 [77][78][79] and PS externalization [80]. In addition, morphological changes in platelets, increased spontaneous whole blood platelet aggregation and decreased platelet life span, as well as elevated plasma platelet factor 3 and augmented urinary excretion of T x A 2 metabolites were all reported in thalassemia [77,81].…”

Section: Oxidative Stress In Plateletsmentioning

confidence: 93%

The Role of Oxidative Stress in Hemolytic Anemia

Fibach¹,

Rachmilewitz

2008

CMM

248

183

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The oxidative status of cells is determined by the balance between pro-oxidants and antioxidants. Pro-oxidants, referred to as reactive oxygen species (ROS), are classified into radicals and nonradicals. The radicals are highly reactive due to their tendency to accept or donate an electron and attain stability. When cells experience oxidative stress, ROS, which are generated in excess, may oxidize proteins, lipids and DNA - leading to cell death and organ damage. Oxidative stress is believed to aggravate the symptoms of many diseases, including hemolytic anemias. Oxidative stress was found in the beta-hemoglobinopathies (sickle cell anemia and thalassemia), glucose-6-phosphate dehydrogenase deficiency, hereditary spherocytosis, congenital dyserythropoietic anaemias and Paroxysmal Nocturnal Hemoglobinuria. Although oxidative stress is not the primary etiology of these diseases, oxidative damage to their erythroid cells plays a crucial role in hemolysis due to ineffective erythropoiesis in the bone marrow and short survival of red blood cells (RBC) in the circulation. Moreover, platelets and polymorphonuclear (PMN) white cells are also exposed to oxidative stress. As a result some patients develop thromboembolic phenomena and recurrent bacterial infections in addition to the chronic anemia. In this review we describe the role of oxidative stress and the potential therapeutic potential of anti-oxidants in various hemolytic anemias.

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Section: Oxidative Stress In Plateletsmentioning

confidence: 93%

The Role of Oxidative Stress in Hemolytic Anemia

Fibach¹,

Rachmilewitz

2008

CMM

248

183

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“…The patients in this study had signs of skin and mucosal hemorrhage, such as bruising easily, epistaxis, and menometrorrhagia [13]. In this study, platelet factor 3 availability, bleeding time, and clot retraction were normal in patients with BTM [13]. After the re-suspension of the platelets of patients with BTM in normal plasma, the reduced aggregation response did not resolve.…”

Section: Discussionmentioning

confidence: 83%

“…In an investigation carried out in 1978, Eldor et al, who took those studies into consideration, observed a reduced platelet aggregation response to collagen, ADP, adrenaline, and ristocetin in patients with BTM and HBT compared to controls. The patients in this study had signs of skin and mucosal hemorrhage, such as bruising easily, epistaxis, and menometrorrhagia [13]. In this study, platelet factor 3 availability, bleeding time, and clot retraction were normal in patients with BTM [13].…”

Section: Discussionmentioning

confidence: 87%

“…In 1963, Hilgartner et al observed signs of skin and mucosal hemorrhage in some patients with beta thalassemia major (BTM), despite the fact that liver parenchymal damage did not occur and clotting factors (factor I, factor II, factor V, factor VII, and factor IX) and prothrombin time was normal [13]. In 1977, Gruppo et al observed that platelets responded to ADP, epinephrine, and collagen with reduced aggregation in patients with sickle cell anemia during vaso-occlusive crisis, and that the platelet factor 3 availability was also high.…”

Section: Discussionmentioning

confidence: 99%

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Could Heterozygous Beta Thalassemia Provide Protection Against Multiple Sclerosis?

et al. 2016

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BackgroundHeterozygous beta thalassemia (HBT) has been proposed to increase the risk of developing autoimmune disease. Our aim in this study was to examine the prevalence of HBT among multiple sclerosis (MS) patients.Material/MethodsHBT frequency was investigated in our MS group (243 patients with MS). Hemoglobin electrophoresis (HE) was carried out if MS patients had a mean corpuscular volume of (MCV) <80 fL and a mean corpuscular hemoglobin level of (MCH) <27 pg/L according to a complete blood count (CBC). If MCV was lower than 80 fL, MCH was lower than 27 pg/L, and Hemoglobin A2 equal to or higher than 3.5%, a diagnosis of HBT was established. The frequency of patients with HBT in our MS patient group was statistically compared with the prevalence of HBT in the city of Istanbul, where our MS patients lived.ResultsThe HBT prevalence was 0.823% (2 patients) in the MS patient group. The prevalence of HBT in Istanbul has been reported to be 4.5%. According to the z-test, the HBT prevalence in our MS patient group was significantly lower than that in Istanbul (Z=6.3611, two-sided p value <0.0001, 95% confidence interval of prevalence of HBT in our MS patient group: 0.000998–0.029413).ConclusionsContrary to our hypothesis at the outset of study, the reduced HBT prevalence in the MS group compared to HBT frequency in the city of Istanbul might indicate that HBT is protective against MS.

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“…Patients with β-thalassaemia are reported to have activated platelets and enhanced platelet aggregation [5] based on the increased expression of the in vivo platelet activation of P-selectin and CD63. Several studies on platelet function investigation in β-thalassaemia major report an in vitro defect of platelet function, when tested by light transmission aggregometry (LTA) [6,7,8,9,10]. We found only one report in the literature concerning the evaluation by PFA-100 platelet function analyser in 14 patients with thalassaemia [11].…”

Section: Introductionmentioning

confidence: 80%

Laboratory Investigation of Platelet Function in Patients with Thalassaemia

Theodoridou

Economou²,

Vyzantiadis³

et al. 2014

Acta Haematol

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The aim of this study was to investigate platelet function in patients with thalassaemia and to detect any relation to chelation treatment (deferasirox or deferiprone/deferiprone plus desferioxamine). Thirty-three transfusion-dependent patients with thalassaemia were included. The investigation consisted of aggregation testing of platelet-rich plasma by light transmission aggregometry (LTA) with the use of 5 agonists as well as the global test of haemostasis by means of the PFA-100 platelet function analyser. In 66.67% of the patients, there was reduced LTA to at least one agonist and in 18.18% there was reduced LTA to two or more agonists. The PFA-100 test was prolonged in 60.6% of the cases. An abnormal LTA and a prolonged PFA-100 time were recorded in 33.3% of the patients and 27.4% had a normal aggregation and PFA-100 test. No correlation between chelation regimen and either LTA or PFA-100 test was found. The abnormal LTA can be explained either by the release of ADP from the haemolysed red blood cells, which leads to defective platelet aggregation, or by the presence of two platelet populations. An in vitro effect without an in vivo impact could be an alternative explanation. In patients with thalassaemia, the reduced LTA and the prolonged PFA-100 closure time could be an in vitro effect and has a close correlation to the bleeding phenotype of each patient.

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Abnormal Platelet Functions in β Thalassaemia

Cited by 34 publications

References 11 publications

The Role of Oxidative Stress in Hemolytic Anemia

The Role of Oxidative Stress in Hemolytic Anemia

Could Heterozygous Beta Thalassemia Provide Protection Against Multiple Sclerosis?

Laboratory Investigation of Platelet Function in Patients with Thalassaemia

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