PULMONARY THROMBOEMBOLISM IN Β-Thalassemia INTERMEDIA: ARE WE AWARE OF THIS COMPLICATION?

Abstract

We describe a case of pulmonary thromboembolism in a 61-year-old woman with beta-thalassemia intermedia and no other risk factors for thrombosis. Thromboembolism was documented by perfusion lung scan. We review the literature on this uncommon complication of thalassemia intermedia and discuss the pathogenesis and treatment options. A significant relationship between pulmonary thromboembolism and pulmonary hypertension in these patients was noted.

Deconstructing sickle cell disease: Reappraisal of the role of hemolysis in the development of clinical subphenotypes

“…11,12,25-36 Pulmonary thrombosis, a common complication of pulmonary hypertension, is seen frequently in these hemolytic disorders, especially following splenectomy. 12,15,27,35,37,38 Splenectomy has been previously suspected to play a role in the development of vasculopathy in otherwise healthy patients, although this point remains controversial. 39,40 In patients with thalassemia, plasma hemoglobin levels preliminarily have been reported to be higher in splenectomized patients than non-splenectomized patients.…”

Optimal management of β thalassaemia intermedia

“…Haemolysis has mainly been associated with splenomegaly; however, recent evidence suggests that haemolysis, along with other factors, is also the hallmark of a hypercoagulable state in TI (Table II) (Ataga et al, 2007;Taher et al, 2008b). Hypercoagulability justifies the high rate of thromboembolic phenomena in patients with TI (Taher et al, 2006b(Taher et al, , 2008b and may explain other complications such as pulmonary hypertension (PHT) with secondary right heart failure (HF) (Aessopos et al, 2001;Taher et al, 2002). Ineffective erythopoiesis and chronic anaemia also lead to an increase in gastrointestinal iron absorption (Taher et al, 2009a), resulting in non-transfusional iron overload (similar to patients with hereditary haemochromatosis), in the liver and less so in the heart (Roghi et al, 2010;Taher et al, 2010e).…”

Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study

“…3 The degree of ineffective erythropoiesis is the primary determinant of the development of anemia, whereas peripheral hemolysis of mature red blood cells (RBCs) remains secondary. 4 Although the first is mainly associated with skeletal complications attributed to compensatory extramedullary hematopoiesis (EMH), 5 the latter has been linked to more severe complications, such as pulmonary hypertension (PHT), 6,7 with secondary heart failure (HF), and thromboembolic phenomena. 8 Moreover, chronic anemia leads to an increase in gastrointestinal iron absorption, 9 resulting in iron overload, which in turn can cause several serious complications, including HF and endocrine abnormalities, such as diabetes mellitus, hypothyroidism, osteoporosis, and hypogonadism.…”