Elevations of Thrombotic Biomarkers in Hemoglobin H Disease

Abstract

Background: Thalassemia is a group of hereditary hemoglobinopathies caused by decreased or absent synthesis of α and/or β globin chains. Studies have shown that hypercoagulability and thrombosis are common clinical symptoms in β-thalassemia, especially β-thalassemia intermedia, but little is known about in α-thalassemia. This study aims to examine phosphatidylserine (PS) levels, platelet activation, and coagulation markers in splenectomized (S) and nonsplenectomy (NS) patients with hemoglobin (Hb) H disease. Methods: The NS group comprised 20 patients (median age 15.0 years, range, 14–16.5 years), and the S group consisted of 11 patients (median age 16.4 years, range, 14–19.9 years) with Hb H disease; the control group consisted of 20 normal subjects. Hematological parameters were collected. Flow cytometry was used to measure PS exposure on red blood cells. The levels of intercellular adhesive molecule (ICAM)-1, tumor necrosis factor α (TNFα), β-thromboglobulin (TG) and prothrombin fragment 1 + 2 (F1.2) were determined using ELISA test kits. Results: Significant increases in the levels of PS, ICAM-1, TNFα, β-TG, and F1.2 were observed in both patient groups compared to normal controls (p < 0.01). Conclusion: This observation indicates blood coagulation, endothelial injury, chronic low-grade inflammation, platelet activation, and thrombin generation are present in Hb H disease; these findings merit further assessment in a larger prospective cohort to establish possible links with thrombotic manifestations.

The correlation of ineffective erythropoiesis biomarkers and development of extramedullary hematopoiesis in patients with thalassemia

“…However, the PS-exposed RBC levels in patients with thalassemia were higher than in normal controls. 10 Previous studies showed significantly elevated levels of PS-exposed RBCs in β-thalassemia/Hb E patients who underwent splenectomy and were associated with pulmonary hypertension. 11 This study demonstrated a correlation between PS-exposed RBCs levels and EMH in patients with thalassemia.…”

Review article inferior vena cava thrombosis: a case series of patients observed in Taiwan and literature review

“…Notably, one of our patients with IVCT was found to have hemoglobin H disease, along with other risk factors, including pregnancy and an IVC filter. Hemoglobin H disease, also known as α-thalassemia intermedia, has been identified as a hypercoagulable state with the demonstration of elevated thrombotic biomarkers [ 64 , 65 ]. Abnormalities in pathologic red blood cells, activated platelets, endothelial damage, and splenectomy are established mechanisms of thalassemia that could contribute to VTE [ 66 ].…”

Guideline for the Management of Conception and Pregnancy in Thalassaemia Syndromes: A British Society for Haematology Guideline

“…28,[94][95][96][97] Patients with a prior history of thrombosis should be managed as per RCOG GTG 37a recommendations for patients with additional risk factors. 98 The pro-thrombotic state in patients with alpha NTDT is less clearly defined, 99,100 and patients should be managed on an individualised risk assessment basis. 98 VTE risk assessment should take place at the initial antenatal visit at 7-12 weeks of gestation (Booking Visit), 28 weeks of gestation and during any admissions, including delivery and postpartum, using a recognised risk assessment tool to guide decisions.…”