Observation of Echinocytosis in Eight Patients: a Phase Contrast and SEM Study

Abstract

We report eight cases of echinocytosis discovered after screening of stained smears. The presence of echinocytes was exceptional in adults and children but was more frequent in neonates. In all cases we confirmed the presence of abnormal red cells by careful examination of the blood in wet preparations observed in phase contrast and after glutaraldehyde fixation and processing for SEM. No discernible common denominator was found to explain the presence of echinocytes in the blood of our patients. No echinocytogenic factor could be demonstrated in the fresh plasma. Echinocytes appeared quickly in patients who received exchange transfusion or transfusion of normal red cells. By contrast, in vitro incubation in the presence of substrates allowing constant ATP level never resulted in the formation of echinocytes when normal red cells were suspended in patient's plasma.

Echinocytic Sensitivity and Deformability of Human Newborn Red Cells

“…In the former, this condition is related to reduced ATP content, and in the latter, to an associated lipoprotein deficiency. The oc currence of spontaneous echinocytosis in newborns does not appear to be the result of ATP depletion, since red cells of newborns have normal ATP levels [6]. In addition spontaneous echinocytosis could not be re lated to the gestational age of the newborns or to any significant alterations in membrane phospholipid composition of red cells from newborns.…”

“…vitamin E deficiency in new borns [17], and severe burns [8]. Of 8 patients with circulating echinocytes we previously studied, 5 were newborn and 2 were infants [6]. In order to determine if this more fre quent occurrence of echinocytosis in new borns was related to an increased sensitivity of newborn red cells to echinocytic shape change, we compared new'born red cells to adult red cells in their ability to undergo this shape transformation.…”