DOI: http://dx.doi.org/10.18203/2349-3933.ijam20171064

Superior sagittal sinus thrombosis in a case of beta thalassaemia major

Syed Md Azad, Anwesha Mukherjee, Rohit Kapoor, Rohit Bannerji, Jaydeb Ray

Abstract


Patients with beta- thalassaemia major have a wide spectrum of clinical manifestations, from transfusion dependency to asymptomatic anaemia. Some may just have incidental finding of anaemia with splenomegaly during physical examination. Most of them require regular blood transfusion and their haemoglobin is maintained at an acceptable level. However they are at risk for multiple complications such as thrombo-embolic events which may be life threatening. We report here a case of beta- thalassaemia major complicated by cerebral sinus thrombosis, who had minimal clinical symptoms and signs at presentation. This case reiterates the importance of increased vigilance in the clinical management of this vulnerable group of patients.

 


Keywords


Beta thalassaemia major, Cerebral venous thrombosis, Headache, Hypercoagulability

Full Text:

PDF

References


deVeber G, Andrew M, Adams C, Bjornson B, Booth F, Buckley DJ, et al. Cerebral sinovenous thrombosis in children. N Engl J Med 2001;345:417-23.

Pignatti BC, Carnelli V, Caruso V, Caruso V, Dore F, De Mattia D, Di Palma A, et al. Thromboembolic events in beta thalassemia major: an Italian multicenter study. Acta Haematol. 1998;99:76-9.

Sebire G, Tabarki B, Saunders DE, Leroy I, Liesner R, Saint-Martin C, et al. Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome. Brain. 2005;128(Pt 3):477-89.

Wong V, Yu YL, Liang RH, Tso WK, Li AM, Chan TK. Cerebral thrombosis in beta-thalassemia/hemoglobin E disease. Stroke. 1990;21:812-6.

de Bruijn SF, de Haan RJ, Stam J. Clinical features and prognostic factors of cerebral venous sinus thrombosis in a prospective series of 59 patients. for the cerebral venous sinus thrombosis study group. J Neurol Neurosurg Psychiatry. 2001;70:105-8.

Masuhr F, Mehraein S, Einhaupl K. Cerebral venous and sinus thrombosis. J Neurol. 2004;251:11-23.

Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood. 2002;99:36-43.

Cappellini MD, Robbiolo L, Bottasso BM, Coppola R, Fiorelli G, Mannucci AP. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. Br J Haematol. 2000;111:467-73.

Joseph L, Fink LM, Hauer-Jensen M. Cytokines in coagulation and thrombosis: a preclinical and clinical review. Blood Coagul Fibrinolysis. 2002;13:105-16.

Wanachiwanawin W, Wiener E, Siripanyaphinyo U, et al. Serum levels of tumor necrosis factor-alpha, interleukin-1, and interferon-gamma in beta(o)-thalassemia/HbE and their clinical significance. J Interferon Cytokine Res. 1999;19:105-11.

Jan Stam, Sebastian de Bruijn, Gabrielle de Veber. Auticoagulation for cerebral sinus thrombosis. Cochrane Database of Systemic Reviews. 2005(4).

Johnson MC, Parkerson N, Ward S, de Alarcon PA. Pediatric sinovenous thrombosis. J Pediatr Hematol Oncol. 2003;25:312-5.

Soleau SW, Schmidt R, Stevens S, Osborn A, MacDonald JD. Extensive experience with dural sinus thrombosis. Neurosurgery. 2003;52:534-44.