Sickle cell trait and stroke in the young adult

Abstract

Summary: Two young patients with sickle cell trait (AS haemaglobinopathy) and ischaemic stroke are reported. The stroke involved the internal carotid artery territory in one and the brainstem in the other. A review of the literature is presented to suggest that the association of sickle cell trait and cerebral infarction is more than coincidental. Haemoglobin electrophoresis should be undertaken routinely in young subjects with ischaemic stroke.

Sickle Cell Trait and Stroke in Children

“…anesthesia and surgery) could be identified. Posterior circulation infarctions occurred in 2 patients [14,15], in all other cases, the anterior circulation was involved.…”

“…in patients who are heterozygous for hemoglobin S in the absence of other hemoglobinopathy). In total, eleven cases of stroke with electrophoretic confirmation of sickle trait have been reported and reviewed in the literature [9][10][11][12][13][14][15]. Only two of these cases involved children: Schenk [10] described a 12-year-old boy who underwent an uncomplicated tonsil lectomy under general anesthesia and within a few days developed a seizure disorder and bilateral cerebral in farcts due to superior sagittal sinus thrombosis, which ultimately resulted in his death.…”

Sickle Cell Disease and Venous Thromboembolism

“…Alli et al15 reported the presence of skull bone infarction crisis and deep vein thrombosis in a boy with homozygous sickle cell anemia (SCA). Also, in individuals with sickle cell trait (SCT) cerebral infarction and stroke is not rare and there are several case reports related to the incidence of stroke in these individuals 16–19. There are few studies from Mediterranean countries reported the occurrence of CVST and stroke in SCD patients.…”

Sudden death due to a haemoglobin variant

“…As people with sickle cell trait have no sickle cells under normal conditions and therefore show no increased hospitalisation or mortality rates compared to people without the trait, it is not widely recognised as a cause of life-threatening illness or death [15]. However, sudden deaths due to sickle cell crises of individuals with sickle cell trait have been described repeatedly [3,4,6,7,8,10,11,12,13,15]. Physical activity with exertional rhabdomyolysis as well as pathological processes such as heat stress, hypoxic stress, viral illness and poor physical condition that may cause hypoxia, acidosis, dehydration, hyperosmolality, hypothermia or elevated erythrocyte 2,3-diphosphoglycerate (DPG) levels, all of which may transform the silent sickle cell trait into a syndrome resembling sickle cell disease and therefore may contribute to development of a sickle cell crisis [10,13,15].…”