Cerebral Vascular Lesions Accompanying Sickle-Cell Anemia

Arena, Jay M.

doi:10.1097/00005053-194001000-00023

Cited by 3 publications

(5 citation statements)

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“…There also is considerable overlap in the histopathology of pulmonary hypertension and ischemic stroke in patients with sickle cell disease. In autopsy studies on patients with sickle cell disease, large vessel intimal hyperplasia, distorted endothelium, and in situ thrombosis have been seen in arteriopathy associated both with stroke [26][27][28][29] and pulmonary hypertension [30,31]. Although sickle cell disease has generally been considered a microvascular disease, it is clear that pulmonary hypertension and cerebrovascular disease in patients with sickle cell disease share a proclivity for large-vessel arteriopathy.…”

Section: Discussionmentioning

confidence: 99%

“…The similarities in epidemiology, physiology, and histopathology between the cerebral vasculopathy and pulmonary vasculopathy are remarkable: both are characterized by intimal and smooth muscle proliferative vasculopathy in a large vessel distribution, both by vasoconstriction, anemia, lack of association with vaso-occlusive crisis frequency, and lack of protection afforded by fetal hemoglobin. These fac- Cerebral arteries [26][27][28][29] Pulmonary arteries [30,31] Thrombosis in situ Cerebral arteries [29,40] Pulmonary arteries [30,31]…”

Section: Discussionmentioning

confidence: 99%

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Cerebrovascular disease associated with sickle cell pulmonary hypertension

et al. 2006

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In patients with sickle cell disease, anemia is a recognized risk factor for stroke, death, and the development of pulmonary hypertension. We have proposed that hemolytic anemia results in endothelial dysfunction and vascular instability and can ultimately lead to a proliferative vasculopathy leading to pulmonary hypertension. Consistent with this mechanism of disease, we now report a case series of six patients with obliterative central nervous system vasculopathy who also have pulmonary hypertension and high hemolytic rate. These patients, identified in the course of a prospective screening study for pulmonary hypertension, presented with neurological symptoms prompting neuroimaging studies. Compared to 164 other patients of similar age in the screened population, those with newly diagnosed or clinically active cerebrovascular disease have significantly lower hemoglobin levels and higher levels of lactate dehydrogenase. A review of the literature suggests that many clinical, epidemiological, and physiological features of the arteriopathy of pulmonary hypertension closely overlap with those of stroke in sickle cell disease, both known to involve proliferative vascular intimal and smooth muscle hypertrophy and thrombosis. These cases suggest that cerebrovascular disease and pulmonary hypertension in sickle cell disease share common mechanisms, in particular, reduced nitric oxide bioactivity associated with particularly high-grade hemolysis. Clinicians should suspect occult cerebrovascular disease in sickle cell patients with pulmonary hypertension. Am.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cerebrovascular disease associated with sickle cell pulmonary hypertension

et al. 2006

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“…Sickle cell anemia may be associated with a variety of serious cerebral manifestations, such as hemiplegia, aphasia, convulsions, stupor or coma (1,2). These symptoms are usually attributed to thromboses of the cerebral vessels produced by sickled erythrocytes, vascular stasis and degenerative changes in the small and medium-size arteries of the central nervous system (3,4).…”

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confidence: 99%

Cerebral Circulation and Metabolism in Sickle Cell and Other Chronic Anemias, With Observations on the Effects of Oxygen Inhalation 1

Heyman¹,

Jl²,

Tw³

1952

J. Clin. Invest.

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“…The first description of stroke associated with sickle cell anemia was by Sydenstricker et al in 1923 (100). They reported a 5‐year‐old boy with sickle cell disease (SCD), hemiparesis, and seizures (100), with similar reports over the next decade (5,11). Collaborative prospective studies have subsequently shown that stroke is a major cause of morbidity and mortality in SCD.…”

Section: Epidemiology Of Stroke In Children and Adults With Sickle Cementioning

confidence: 77%

“…Early autopsy studies in patients with SCD who suffered stroke showed gradual tapering and complete closure of the large cerebral arteries that was associated with intimal hyperplasia (5,11). In the 1970s and 1980s, angiography was performed in patients with SCD who had experienced overt strokes and demonstrated irregularities of the lumen, with narrowing or total occlusion of the large cerebral vessels, further supporting the previous autopsy findings (36,80,94).…”

Section: Pathologic and Radiologic Findings Of Cerebrovascular Lesionsmentioning

confidence: 99%

Pathophysiology of Stroke in Sickle Cell Disease

Hillery

Panepinto

2004

Microcirculation

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Stroke affects both motor and cognitive function in patients with sickle cell disease (SCD). Symptomatic stroke is associated with intimal disease of the large cerebral arteries. Silent stroke, defined as cerebral infarction in the absence of overt clinical neurologic symptoms, is often due to microinfarcts suggestive of microvascular disease. While the natural history of stroke in SCD is well described, the pathophysiology remains poorly understood and probably varies with the site of vascular injury. Increased red cell adhesion, oxidative injury of the vessel wall, inflammation, abnormal vasomotor tone regulation, and increased activity of the coagulation system all may contribute to cerebral vasopathology in SCD.

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Cerebral Vascular Lesions Accompanying Sickle-Cell Anemia

Cited by 3 publications

References 0 publications

Cerebrovascular disease associated with sickle cell pulmonary hypertension

Cerebrovascular disease associated with sickle cell pulmonary hypertension

Cerebral Circulation and Metabolism in Sickle Cell and Other Chronic Anemias, With Observations on the Effects of Oxygen Inhalation 1

Pathophysiology of Stroke in Sickle Cell Disease

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