Abstract
A 10-yr-old HbE/Beta thalassemia child who developed subacute to chronic occipitotemporal hemorrhagic infarct with smaller chronic infarct with gliotic changes in the left frontal periventricular white matter. Genetic tests showed that patient was positive for HbE and IVS1-5 mutation and was negative for thrombogenic mutations. Hemorrhagic infarct was confirmed by magnetic resonance imaging study. Antigenic levels of Protein C and Protein S were low. Based on these outcomes, it was concluded that Protein C and Protein S deficiency were the causative factor for developing hemmoragic infarct in the HbE/ Beta thalassemia patient.
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References
Rees DC, Styles L, Vichinsky EP, Clegg JB, Weatherall DJ. The hemoglobin E syndromes. Ann N Y Acad Sci 1998; 850: 334–343.
Capellini MD, Robbiolo L, Bottasso BM, Cappola R, Fiorelli G et al. Venus Thromboembolism and hypercoagulability in splenectomized patients with thalassemia intermedia. Br J Hematol 2000; 111: 467–473.
Logothetis J, Constantoulakis M, Economidou J, Stefanis C, Hakas P, Augoustaki O et al. Thalassemia major: A survey of 138 cases with emphasis on neurological and muscular aspects. Neurology 1972; 22: 294–304.
Borgna Pignatti C, Carnelli V, Caruso V et al. Thromboembolic events in beta thalassemia major: an Italian multicenter study. Acta Haematol 1998; 99: 76–79.
Paolino E, Monetti VC, Granieri E, Boldrini. Acute cerebrovascular insults in homozygous beta-thalassaemia: a case report. J Neurol 1983; 230: 37–41.
Wong Virginia, Yu YL, Liang RHS, Tso WK, Li AMC, Chan TK. Cerebral Thrombosis in β-Thalassemia/Hemoglobin E Disease. Stroke 1990; 21: 812–816.
Winichagoon P, Fucharoen S, Wasi P. Increased circulating platelet aggregates in thalassemia. Southeast Asian J Trop Med Public Health 1981; 12: 556–560.
Sonakul D, Pacharee P, Laohapand T, Fucharoen S, Wasi P. Pulmonary artery obstruction in thalassemia. Southeast Asian J Trop Med Public Health 1980; 11: 516–523.
Fucharoen S, Youngchaiyud P, Wasi P. Hypoxaemia and the effect of aspirin in thalassemia. Southeast Asian J Trop MedPublic Health 1981; 12: 90–93.
Ralph LS, Owen J, Mohr JP, Thomas KT, Betty AG. Free Protein S Deficiency: A Possible Association With Cerebrovascular Occlusion. Stroke 1989; 20: 1657–1661
Aysun KB, Yurdanur K. Natural coagulation inhibitors (protein C, protein S, antithrombin) in patients with sickle cell anemia in a steady state. Pediatrics International 2001; 43: 592–596.
Eldor A, Rachmilewitz AE. The hypercoagulable state in thalassemia. Blood 2002; 99: 36–43.
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Vineeta, S., Biswas, A., Kumar, B. et al. Protein C and Protein S: Causative factor for developing a hemorrhagic infarct in a HbE/Beta thalassemia child. Indian J Pediatr 77, 316–317 (2010). https://doi.org/10.1007/s12098-009-0297-5
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DOI: https://doi.org/10.1007/s12098-009-0297-5