Altered vascular reactivity in sickle hemoglobinopathy. A possible protective factor from hypertension

Patients with sickle cell anemia have considerably less hypertension than the black population in general. Factor(s) offering "protection" from hypertension in these patients remain unknown. Hormonal and hemodynamic parameters involved in blood pressure regulation were evaluated in normotensive, stable sickle cell patients and black nonsickle normotensive controls. There was no difference in systolic, diastolic, or mean arterial blood pressure between the two groups. The characteristic hemodynamic findings of increased cardiac index, renal plasma flow, and plasma volume were observed in the sickle cell patients. Urinary sodium excretion was comparable on an ad libitum, high sodium, and low sodium diet. In contrast, plasma renin activity was greater in sickle cell patients at all levels of sodium intake in both supine and upright positions. These findings suggested possible altered vascular responsiveness to endogenous angiotensin II. Plethysmography revealed that sickle cell patients had greater forearm blood flow than normal controls and black nonsickle chronic anemic controls at rest, during cold stimulation, and during exercise. Forearm vascular resistance was significantly lower in the patients and did not increase with cold-induced, sympathetic-mediated stimulation. To assess these findings more directly, the pressor response to the exogenous administration of graded doses of angiotensin II and norepinephrine was measured. There was a marked decrease in the pressor response to angiotensin II but not to norepinephrine in the sickle cell patients. The findings in these studies indicate fundamental differences in blood pressure control in the sickle cell patient, probably at the vascular level.