Silent cerebral infarct in child patients with beta thalassaemia intermedia

Abstract

There are numerous reports that patients with thalassemia are faced with hypercoagulability leading to vascular disorders. One of these complications is known as a silent infarct, defined as a small infarct detected by cerebral imaging but without any neurological symptoms. Since it has a progressive nature, it is of vital importance because it may lead to symptomatic cerebrovascular accidents in the future. Twenty-two children with thalassemia intermedia were enrolled into the study and MRI scans were performed. All demographic data and clinical features of the patients were obtained during the follow-up period. In addition to the patients, 13 healthy controls were included to compare serum anticoagulant levels with those of the thalassemia intermedia patients. Four of the patients were found to have silent cerebral infarcts (SCIs). The lesions involved varying amounts of the deep cerebral white matter and sub-cortical areas. One patient showed 'net line' filling defects within the ambient cistern on MRI images corresponding to moyamoya vessels. Three patients had undergone splenectomy, and three were transfused irregularly and had less than six transfusions per year. More importantly, protein C levels were lower and platelet levels were significantly higher in the patient group compared with controls. We were not able to find any association between SCI and transfusion number or splenectomy. However, of the total patients four thalassemia intermedia patients had SCI in early childhood and this is an unusual finding. In order to verify the findings, further studies must be conducted involving larger numbers of patients.

Modified Magnetic Resonance Imaging Burden of Cerebral Small Vessel Disease and Related Risk Factors in Patients With Thalassemia

“…25 Our study also revealed that aging is an independent risk factor for the total burden of CSVD and all single markers except RSSI. Other risk factors for cerebral ischemia in patients with thalassemia have been reported, such as increased white blood cell count, 4 splenectomy, 5,13 decreased protein C level, increased platelet level, 7 and increased serum ferritin, 12 which were not found in our study.…”

“…The incidence of SCI in patients with thalassemia ranges from 18% to 60% 3–5,7–10 . Most of the lesions are lacunar and single, and the distribution is uncertain, involving all lobes, basal ganglia, deep white matter, and infratentorial area.…”

“…The incidence of SCI in patients with thalassemia ranges from 18% to 60%. [3][4][5][7][8][9][10] Most of the lesions are lacunar and single, and the distribution is uncertain, involving all lobes, basal ganglia, deep white matter, and infratentorial area. However, most of the literature includes retrospective studies Of the 18 patients with SCI, 3 had evidence of arterial stenosis on magnetic resonance angiography, whereas only 1 had a cerebral infarction after the supply area of the diseased blood vessels.…”

“…Thrombosis is one of its complications, including deep vein thrombosis, cerebral vascular thrombosis, portal vein thrombosis, and pulmonary embolism . Most of the ischemic changes in patients with thalassemia include silent cerebral infarction (SCI) or white matter lesions, 3–14 suggesting that most of them may be cerebral small vessel disease (CSVD).…”

Review of disease-related complications and management in adult patients with thalassemia: A multi-center study in Thailand

“…[ 6 – 10 ] Thrombosis, pulmonary hypertension, right ventricular disorders, and silent cerebral infarction are more common in patients with NTDT. [ 11 – 14 ] Moreover, a specific clinical risk factor, splenectomy, is a strong risk factor for pulmonary hypertension and thrombosis in patients with thalassemia. [ 15 – 18 ] Tailoring treatment based on patient specific risk factors should be the optimal management in patients with thalassemia in a country with limited resources.…”