Thromboembolic events have been frequently reported in ß-thalassemic patients. The incidence of these events has been reported to be from 1.1-5.3% in thalassemia major patients.
1 The hypercoagulable state in thalassemia increases the risk of venous thromboembolic events in deep veins of limbs, portal vein, cerebral veins and central venous lines.
2 There are various factors which contribute to hypercoagulable state in thalassemia including platelet abnormalities, defect in red cell membrane, oxidant injury, defects in coagulation inhibitors.
2 Splenectomy and transfusion naveity are important risk factors for thrombotic events, especially in patients with thalassemia intermedia.
3 Portal vein thrombosis has rarely been reported in patients with thalassemia major.
1,4,5 Various clinical studies have shown that splenectomised patients have a higher incidence of thromboembolic events than non-splenectomized patients.
5 Our patient was a female with a huge splenomegaly but developed portal cavernoma before splenectomy. Thromboembolic events in thalassemia usually manifest in second or third decade of life. Our patient developed symptoms at a very young age. They usually present with symptoms of increased portal venous pressure such as gastrointestinal bleeding and abdominal pain.
4 Similarly our patient presented with hematemesis. To diagnose this condition, many methods such as ultrasound and color doppler, CT scan, angiography, are useful.
4 We diagnosed our patient to have portal cavernoma with ultrasound and doppler of abdomen. Transfusion therapy may be beneficial to prevent the occurrence of thrombotic events.
3 Early diagnosis and treatment with thrombolytic agents in acute portal vein thrombosis can be helpful. Splenectomy in patients with ß-Thallesemia should be performed only when it is strongly indicated. If splenectomy has to be performed anticoagutant prophylaxis should be instituted.
4 Our patient was on regular blood transfusions and had features of hypersplenism; and hence underwent splenectomy with proximal lienorenal shunt. She received low molecular weight
Heparin post splenectomy for 10 months, however her latest Doppler shows non-visualisation of the shunt. Since splenectomy is a major factor causing thromboembolic events in thalassemia patients, reassessment of the procedure and risk benefit-evaluation is necessary.
3 Similarly, our patient continued to have hematemesis post splenectomy and whether that is an additional contributing factor to the persistent portal hypertension needs to be assessed, though there is no portal thrombosis noted.
| References : |
- Sadeghi S, Mohammadi Ashiani A, Khalili M. Portal Vein Thrombosis Following Splenectomy in β-thalassemia Major. IJBC 2016; 8(3): 90-91.
- Thromboembolic complications in b-thalassemia: beyond the horizon. Panigrahi I, Agarwal S. Thrombosis Research (2007); 120:783-789.
- Thalassemia and Venous Thromboembolism. Succar J, Musallam KM, Taher AT. Mediterr J Hematol Infect Dis 2011; 3; Open Journal System.
- Hassan MN, Tahereb GM, Ahmad T, Asghar DA, Reza ED, Ali B, Mohsen MN. Correlation of splenectomy with portal vein thrombosis in beta-thalassemia major. J Pak Med Assoc. 2011 Aug;61(8):760-2. PMID: 22355997.
- Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, Saned M, Cesaretti C, Cappellini MD. Splenectomy and thrombosis: the case of thalassemia intermedia. J Thromb Haemost 2010; 8: 2152-8.
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