Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia

Abstract

Thromboembolic phenomena have been described in patients with thalassaemia intermedia and major, although there are relatively few epidemiological data on the overall frequency of these complications. To obtain more insight into the risk and mechanism of venous thromboembolism in thalassaemia, the aims of this study were: (i) to establish retrospectively the prevalence of thromboembolic events in a large group of adults with thalassaemia intermedia and major during a follow up period of 10 years; (ii) to measure in subgroups of these patients sensitive markers of activation of coagulation and fibrinolysis enzymes; and (iii) to look for possible procoagulant mechanisms. A high prevalence of thromboembolic events was found, particularly in splenectomized patients with thalassaemia intermedia (29%). These patients had high plasma levels of markers of coagulation and fibrinolysis activation. Furthermore, thalassaemic red cells and erythroid precursors from splenectomized patients with thalassaemia intermedia had an enhanced capacity to generate thrombin. To evaluate the role of splenectomy per se on procoagulant activity, we evaluated the capacity to form thrombin in healthy individuals who had been splenectomized for trauma. They produced the same amount of thrombin as non‐splenectomized controls. In conclusion, the results of this study show the existence of a hypercoagulable state in splenectomized patients with thalassaemia intermedia and that their red and erythroid cells are capable of acting as activated platelets in thrombin generation.

Thrombosis of the splenoportal axis after splenectomy

“…Myeloproliferative disorders are usually associated with massive splenomegaly and thrombocytosis and these factors have been related to the increased risk for PSVT development. Regarding hemolytic anemia, only one report demonstrated its association with risk of thrombosis [25] and in our experience, it should be considered a significant risk factor. As hemolytic anemia is not necessarily associated with splenomegaly and thrombocytosis, this disease could be considered an independent risk factor.…”

Thalassemia and risk of dementia: A nationwide population-based retrospective cohort study

“…In addition, a profound hemostatic change with a hypercoagulable state has also been recognized in patients with β-thalassemia major, β-thalassemia intermedia, and α-thalassemia (hemoglobin H disease) [27]. Some studies have also primarily reported cerebral thrombotic events in patients with β-thalassemia, and other thromboembolic events including deep venous thrombosis, pulmonary embolism, silent cerebral infarction, or recurrent arterial occlusion observed in serial studies [28][29][30]. Therefore, these effects, including vascular injury, anemia, and a hypercoagulable state related to silent stroke or small vessel disease may increase the risks of developing long-term dementia in thalassemia patients.…”

Intravascular Hemolysis, Vascular Endothelial Cell Activation and Thrombophilia in Splenectomized Patients with Hemoglobin E/β-Thalassemia Disease

“…Indications for splenectomy are hypersplenism, increased blood transfusion requirement, and physical encumberment [1]. A well-known complication of splenectomy is an increased risk of infection [3,4], and with longer survival rates after splenectomy in patients with thalassemia intermedia, an increased incidence of venous thromboembolism and hypercoagulability was reported [5]. …”