Abnormal erythrocyte endothelial adherence in hereditary stomatocytosis
BD Smith and GB Segel
Department of Medicine, University of Rochester School of Medicine, NY
14620, USA.
Hereditary stomatocytosis is a red cell membrane protein disorder, which
results in hemolytic anemia. Some patients with hereditary stomatocytosis
experience dyspnea, chest pain, and abdominal pain, particularly after
splenectomy. These symptoms may represent vaso- occlusion secondary to
adherence of an abnormal erythrocyte membrane to vascular endothelium. We
studied three members of a family with varying clinical expression of
hereditary stomatocytosis. Adherence of red cells to endothelium was
quantified by measuring the shear force required to separate individual
cells from endothelial monolayers using a micropipette technique. Two
patients with symptoms of in situ thromboses had a higher percentage of
adherent cells compared with their asymptomatic sibling and normal
controls. Correlation between this in vitro phenomenon and the clinical
course suggests that flow abnormalities in the microcirculation
attributable to erythrocyte endothelial adherence may play an important
pathogenetic role in the illness. When the proportion of adherent red cells
was reduced by a chronic transfusion program in one patient and
pentoxifyllin therapy in another, the vaso-occlusive complications were
eliminated.
Volume 89,
Issue 9,
pp. 3451-3456,
05/01/1997
Copyright © 1997 by The American Society of Hematology
