Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease

Abstract

Stroke is a relatively frequent and severe complication of sickle cell disease. We performed cerebral arteriograms in 30 patients with sickle cell disease to evaluate the cause of acute neurologic deficits and to assess the effects of transfusion therapy given for a year or more after the acute episode. Twenty-three patients with motor and speech deficits had multiple abnormalities of major cerebral arteries. The internal carotid and anterior and middle cerebral arteries showed stenosis and/or occlusion at their common junction. Irregular luminal surfaces suggested that endothelial damage and intimal hyperplasia were the basis of stroke. Prolonged transfusion therapy nearly stopped progression of stenosis and markedly decreased the irregularity of the luminal surfaces; in 4 untransfused patients, the degree of stenosis doubled and the luminal abnormalities persisted. Prior to transfusion, 90% of patients had recurrence of stroke. With transfusion therapy, only 10% of patients had recurrence despite persistent arterial abnormalities. Clinical recurrences per patient-month decreased 75- fold. The patients tolerated prolonged transfusion therapy well, despite progressive iron accumulation. Seven patients with smooth abnormalities of a single artery, nonocclusive changes, or with normal arteriograms did not receive transfusions. Only one of this group had recurrence of symptoms.

Management Of Cerebral Vasculopathy In Children With Sickle Cell Anaemia

“…The frequency of recurrent clinical stroke was reduced by 90% in some reports (Buchanan et al, 1983). Repeat angiograms showed absence of progression of vessel disease and occasional smoothing of previously roughened endothelium in some patients, but progression of large-vessel disease in others (Russell et al, 1984). Serial MRA con®rms a similar variability in response.…”

“…There is an unexplained association of priapism to cerebral ischaemia (Rackoff et al, 1992). An SS family member with a stroke is a strong predictor of stroke (Powars et al, 1984(Powars et al, , 1990Russell et al, 1984;Adams et al, 1994). Haplotype of the b S gene cluster (CAR) and a gene status are important correlates of severe disease expression and stroke risk, but should not be taken as the sole determinants for initiation of therapy (Powars et al, 1990;Adams et al, 1994;Figueiredo et al, 1996).…”

“…Transfusion therapy. Transfusion therapy with type-speci®c Hb AA red cells is the sole therapeutic modality that has shown consistent clinical ef®cacy in SS subjects with cerebrovascular disease (Sarnaik et al, 1979;Russell et al, 1984). The goal is to maintain the Hb concentration at 12 g/dl and the Hb S percentage at < 30%.…”

“…Transfusion after clinical stroke. The original encouraging studies of transfusion therapy (Sarnaik et al, 1979;Russell et al, 1984) were performed in SS children after an overt clinical stroke. The frequency of recurrent clinical stroke was reduced by 90% in some reports (Buchanan et al, 1983).…”

Transcranial Doppler ultrasonography in siblings with sickle cell disease

“…Genetic or environmental risk factors may influence the risk of stroke in SCD. Anecdotal reports have described the occurrence of stroke in multiple family members with SCD (Russell et al, 1984), and a recent sibling pair analysis more formally demonstrated an increased risk of stroke in siblings with SCD (Driscoll et al, 1997), supporting the presence of either genetic or environmental risk factors. Different human leucocyte antigen (HLA) genotypes have also been associated with either an increased or decreased risk of stroke in SCD (Styles et al, 2000), further suggesting the existence of genetic modifiers.…”