Intracranial hemorrhage and circulating coagulation inhibitor in β-thalassemia major

Sinniah, D.; Ekert, H.; Bosco, John J.; Nathan, Luciana Piovesan; Koe, Stuart

doi:10.1016/s0022-3476(81)80387-3

Cited by 11 publications

(5 citation statements)

References 6 publications

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“…The clinical manifestations and the pathological find ings in our patient arc clearly distinct from those previ ously described by these investigators [13][14][15], The most striking finding was the close temporal relationship be tween the blood transfusion leading to a pronounced (50%) increase in hematocrit and the development of a permanent neurologic damage. This sequence of events is reminiscent of a single patient described by Logothetis et al [13] in whom stroke appeared shortly after a series of intensive transfusions.…”

Section: Discussionsupporting

confidence: 64%

“…These were attrib uted to the presence of vasopressive substances presum ably provided by multiple blood transfusions. Sinniah et al [15] described 4 thalassémie patients with similar manifes tations to those described by Wasi et al [14] in whom a cir culating prothrombinase inhibitor was identified, causing marked prolongation of the prothrombin and partial thromboplastin times [15].…”

Section: Discussionmentioning

confidence: 70%

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Thromboembolic Complications in Beta Thalassemia major

et al. 1992

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Thromboembolic complications in β-thalassemia major have seldom been reported and their association with risk factors such as left ventricular failure and postsplenectomy thrombocytosis has remained speculative. In this report we describe 4 patients with unusual thromboembolic manifestations: recurrent arterial occlusion, recurrent pulmonary thromboembolism, venous thrombosis and a fatal cerebrovascular event. Although in all patients both risk factors were present, the precise causes for their thromboembolic complications were not identified. In 1 patient, however, a marked increase in hematocrit following blood transfusion resulted, in all likelihood, in a fatal cerebrovascular infarction. We suspect that these patients (constituting 4% of our β-thalassemic group) represent a subset of those with high susceptibility to both arterial and venous thromboembolic complications.

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Section: Discussionsupporting

confidence: 64%

Section: Discussionmentioning

confidence: 70%

Thromboembolic Complications in Beta Thalassemia major

et al. 1992

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“…It is well known that some patients with /3-thalas semia major after several transfusions develop some vascular CNS lesions [1][2][3][4][5] without significant hemo static alteration. Several kinds of lesions have been re ported.…”

Section: Introductionmentioning

confidence: 99%

“…Some authors [1,3] have observed that imme diately after blood transfusions, some thalassémie pa tients showed neurological sequelae secondary to the thrombosis of cerebral vessels. In some patients main tained on low values of hemoglobin (Hb), other au thors [2,4,5] found characteristic syndromes like hy pertension, headache, convulsions, deficit of cranial nerves and cerebral hemorrhage when Hb levels were enhanced by transfusion. In an attempt to explain these alterations, studies of coagulation factors have been performed in thalassémie patients [1,2,6,7], A reduction of coagulation factors XI and XII was found.…”

Section: Introductionmentioning

confidence: 99%

“…In some patients main tained on low values of hemoglobin (Hb), other au thors [2,4,5] found characteristic syndromes like hy pertension, headache, convulsions, deficit of cranial nerves and cerebral hemorrhage when Hb levels were enhanced by transfusion. In an attempt to explain these alterations, studies of coagulation factors have been performed in thalassémie patients [1,2,6,7], A reduction of coagulation factors XI and XII was found. This indicated an activation of the intrinsic system, while the alteration of the vitamin-K-dependent factors was not appreciable.…”

Section: Introductionmentioning

confidence: 99%

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Protein C and Antithrombin III in Polytransfused Thalassemic Patients

et al. 1987

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Seventy-four patients with β-thalassemia major were studied to test the hypothesis that a deficiency of protein C (PC) and antithrombin III (AT III), both antithrombotic proteins, could contribute to the pathogenesis of CNS thromboembolic lesions. In 70 patients, PC levels were found to be significantly lower than normal, whereas AT III activity was found to be lower only in 41 patients. The lowest values of PC and AT III were found in older splenectomized patients, a low PC value only was found in chronic hepatitis patients. Prothrombin time and fibrinogen were found to be particularly abnormal in patients with chronic hepatitis and without spleen. A relatively poor correlation was observed between PC and AT III (p < 0.02). PC correlated with age (p < 0.001), transfusional iron (p < 0.001) and ferritin (p < 0.001). It also correlated with serum albumin (p < 0.001), prothrombin time (p < 0.001) and fibrinogen (p < 0.02) and with serum transaminases (GPT) (p < 0.001). The same indexes correlated less significantly with AT III activity. Nevertheless, only 2 of our patients had CNS thromboembolic complications. It is probable that low clotting factors, hyperfibrinolysis and thrombocytopenia (which are common in chronic liver disease) could have the opposite effect on hemostasis from that of low levels of anticoagulant proteins such as PC and AT III.

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Cerebral infarction in infants and children: Clinical features, CT and EEG findings

Azam

1998

Indian J Pediatr

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In a retrospective study, medical records of twenty one infants and children who were diagnosed for cerebrovascular disease, were analysed. Patients with history of head injury, brain tumor and intracranial infections were excluded from the study. Age ranged between six weeks and 12 years. Fourteen were boys and 7 girls. Ten patients (47%) had known underlying risk factor. Hemiparesis and seizures were commonest presenting features as well as long term neurological sequelae in survivors. CT scan was abnormal in all patients whereas, EEG was abnormal in 90% with 76% lateralizing effect.

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Intracranial hemorrhage and circulating coagulation inhibitor in β-thalassemia major

Cited by 11 publications

References 6 publications

Thromboembolic Complications in Beta Thalassemia major

Thromboembolic Complications in Beta Thalassemia major

Protein C and Antithrombin III in Polytransfused Thalassemic Patients

Cerebral infarction in infants and children: Clinical features, CT and EEG findings

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