The thrombotic spectrum of B-thalassemia

Vasilopoulou, Mavra; Stafylidis, Christos; Politou, Marianna

doi:10.1016/j.tru.2022.100102

Cited by 5 publications

(5 citation statements)

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“…The leading causes of death are heart disease, diabetes, hepatocellular carcinoma, and liver disease. 18,22 Indrani comparable outcomes, too. Patients with thalassemia have been shown to have elevated levels of malondialdehyde and other plasma lipid peroxidation indicators.…”

Section: Discussionmentioning

confidence: 73%

Clinical Significance of Serum Iron in the Diagnosis of Iron Deficiency Anemia in Patients from Local Population of Punjab-Lahore

Farheen¹,

Malik²,

Anwar³

et al. 2023

PJMHS

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Background: The main cause of anemia in one-third of the world's population is a lack of iron (ID). Anemia affects between 700 and 800 million individuals globally. Methodology: Only the patients diagnosed with iron deficiency anemia was selected from Ali Fatima Hospital Lahore and Jinnah Hospital Lahore for determination of serum iron concentration in comparison with healthy controls. Results: In our study male patients were 67% and females were 33%. Frequency of children with beta thalassemia major under 5 year of age is 19%, 5-9 year is 24%, 9-15 years is 31% and above 15 years is 26% Practical implications: The current study aims to spread the awareness among community about clinical significance of serum iron in the diagnosis of iron deficiency anemia in patients and ways to control the iron deficiency. This investigation will assist in determining the severity of the condition in our community as well as the status of serum iron levels in individuals with iron deficiency anemia. The evaluation of serum iron levels in anemic individuals will be helpful to medical experts in determining the best course of treatment. Conclusion: We concluded that renal tubular damage and hepatocellular injury may be secondary to oxidative lipid peroxidation mediated by the iron overload. However further investigations are extremely necessary for this interpretation. A better evaluation of iron toxicity and regular monitoring of renal functions, liver functions, accompanied by an improvement in chelation therapy along with the administration of selective antioxidants (vitamins E and C), may protect different organs against oxidative stress and its consequences, specifically endocrine problems. Keywords: Anemia, Iron deficiency, Ferritin, Hemoglobin, Thalassemia, hepatocellular injury.

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Section: Discussionmentioning

confidence: 73%

Clinical Significance of Serum Iron in the Diagnosis of Iron Deficiency Anemia in Patients from Local Population of Punjab-Lahore

Farheen¹,

Malik²,

Anwar³

et al. 2023

PJMHS

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“…In recent years, several studies reported an increase in the haemostatic derangement complication rates among β‐thalassemia major patients 4–6 . This derangement ranges from subclinical abnormalities of haemostatic parameters to severe hypercoagulability and thromboembolism.…”

Section: Introductionmentioning

confidence: 99%

“…This derangement ranges from subclinical abnormalities of haemostatic parameters to severe hypercoagulability and thromboembolism. It has been reported that thrombosis tendency in thalassemia patients may be related to frequent transfusion, iron overload, diabetes, liver disease, splenectomy, and presence of elevated levels of endothelial adhesion proteins such as intercellular adhesion molecule‐1 (ICAM‐1), E‐selectin (ELAM‐1), vascular cell adhesion molecule‐1 (VCAM‐1), von Willebrand factor (vWF), and thrombomodulin 4,7 . All of these factors were found to increase platelet activation, endothelial activation, and alter coagulation protein level in blood 8 …”

Section: Introductionmentioning

confidence: 99%

“…1 In recent years, several studies reported an increase in the haemostatic derangement complication rates among β-thalassemia major patients. [4][5][6] This derangement ranges from subclinical abnormalities of haemostatic parameters to severe hypercoagulability and thromboembolism. It has been reported that thrombosis tendency in thalassemia patients may be related to frequent transfusion, iron overload, diabetes, liver disease, splenectomy, and presence of elevated levels of endothelial adhesion proteins such as intercellular adhesion molecule-1 (ICAM-1), E-selectin (ELAM-1), vascular cell adhesion molecule-1 (VCAM-1), von Willebrand factor (vWF), and thrombomodulin.…”

Section: Introductionmentioning

confidence: 99%

“…It has been reported that thrombosis tendency in thalassemia patients may be related to frequent transfusion, iron overload, diabetes, liver disease, splenectomy, and presence of elevated levels of endothelial adhesion proteins such as intercellular adhesion molecule-1 (ICAM-1), E-selectin (ELAM-1), vascular cell adhesion molecule-1 (VCAM-1), von Willebrand factor (vWF), and thrombomodulin. 4,7 All of these factors were found to increase platelet activation, endothelial activation, and alter coagulation protein level in blood. 8 Von Willebrand factor (vWF) protein is a large multimeric molecule that interacts with platelet glycoproteins forming a complex compound, causing platelet aggregation and adhesion at the site of injury.…”

Section: Introductionmentioning

confidence: 99%

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Evaluating von Willebrand factor and ADAMTS13 levels in thalassemia major patients and assessing a possible association with Thrombospondin‐1

Al‐Sabaan,

Al‐Awadhi

2023

Int J Lab Hematology

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IntroductionAlterations in the endothelium and endothelial adhesion proteins such as von Willebrand factor (vWF) play major roles in hypercoagulability in thalassemia. vWF protein release leads to platelet aggregation and thrombi formation at the site of vascular injury. It is then degraded by the proteolytic enzyme ADAMTS13. Thrombospondin‐1 is a multifactorial glycoprotein, which was reported to compete with ADAMTS13 for sites of vWF proteolysis. In this study, levels of vWF, ADAMTS13, and TSP‐1 proteins were determined in β‐thalassemia major patients. A possible association between TSP‐1 and vWF and ADAMTS‐13 was also evaluated.MethodsThe study was conducted on 80 β‐thalassemia major patients and 80 age and sex matched healthy controls. The 80 patients were sub‐divided into two groups; splenectomised and non‐splenectomised. vWF, ADAMTS13 and TSP‐1 plasma level were measured using ELISA technique.ResultsThere was no significant difference in vWF and TSP‐1 levels between patients and controls (p > 0.05). However, ADAMTS13 levels and ADAMTS13 activity/vWF antigen ratio were significantly higher in patients compared to controls (p < 0.05). VWF antigen and TSP‐1 level were significantly higher in splenectomised patients (p = 0.025 and p < 0.001, respectively). We also observed a significant decrease in ADAMTS13 activity/vWF antigen ratio among splenectomised compared to non‐ splenectomised patients (p = 0.019). Correlation analysis showed a significant negative correlation between TSP‐1 and vWF Collagen Binding Activity (r = −0.394, p = 0.021) and a positive correlation with ADAMTS13 activity/vWF antigen ratio (r = 0.356, p = 0.039) in splenectomised compared to non‐ splenectomised patients.ConclusionOur findings highlight the adequacy of patient management protocols for β‐TM in Kuwait as patients presented with comparable levels of platelets, vWF and TSP‐1 compared to normal controls. The reported increase in ADAMTS13 in patients may be required to maintain normal levels of vWF. Although no active thrombotic episodes were reported at the time of the study, the significant rise in platelets, vWF:Ag and TSP‐1 levels in splenectomised patients may indicate a tendency towards hypercoagulability. Monitoring of splenectomised patients is recommended.

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溶血性貧血と血栓症

Ueda¹

2022

Nihon Kessen Shiketsu Gakkaishi

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The thrombotic spectrum of B-thalassemia

Cited by 5 publications

References 64 publications

Clinical Significance of Serum Iron in the Diagnosis of Iron Deficiency Anemia in Patients from Local Population of Punjab-Lahore

Clinical Significance of Serum Iron in the Diagnosis of Iron Deficiency Anemia in Patients from Local Population of Punjab-Lahore

Evaluating von Willebrand factor and ADAMTS13 levels in thalassemia major patients and assessing a possible association with Thrombospondin‐1

溶血性貧血と血栓症

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