Abstract
Investigation of a patient with sickle cell disease (SS) and hemoglobinuria led us to realize that SS patients may develop exercise-induced hemoglobinemia. 6 patients: 4 SS and 2 Hgb SC (SC) were studied before and after exercise. All had plasma Hgb <5ng% initially. After exercise plasma Hgb in SS patients rose 10-16 mg% but remained <5 mg% in SC. To see if patient's RBCs were abnormally fragile when exposed to shear forces that could be generated in the small vessels of exercising muscles, we exposed them to physiologic shear rates in a coneplate viscometer (Hct 70, 37°C, pO2 100) for 2 min. Normal and SC RBCs had no hemolysis at shear stresses less than 2000 dynes/cm2 (d/cm2) and 1% at 2300 d/cm2. Hemolysis of SS RBCs began at 700 d/cm2 and continued linearly to 7% at 2300 d/cm2. This shear/hemolysis profile is comparable to that of xerocytes and artificially dehydrated RBCs. SS RBCs separated on Stractan gradients had populations of dehydrated cells that were increasingly shear fragile with increasing dehydration. The abnormal shear/hemolysis profile of these dehydrated layers was normalized by in vitro rehydration using nystatin and a high K buffer. Such dense and fragile layers were not found in SC or normal blood. These data suggest that the exercise-induced hemolysis in SS patients is related to lysis of dehydrated, shear-sensitive cells. This same process may also contribute to the chronic hemolysis of SS - a phenomenon known to correlate with numbers of dehydrated, irreversibly sickled cells.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Platt, O., Lux, S. & Nathan, D. 851 EXERCISE-INDUCED HEMOLYSIS IN SICKLE CELL DISEASE. Pediatr Res 15 (Suppl 4), 584 (1981). https://doi.org/10.1203/00006450-198104001-00876
Issue Date:
DOI: https://doi.org/10.1203/00006450-198104001-00876