RED-CELL AND PLASMA LIPIDS IN ACANTHOCYTOSIS*

Abstract

The rare syndrome of acanthocytosis was first described in 1950 by Bassen and Kornzweig (1) and two years later by Singer, Fisher, and Perl- stein (2). The principal manifestations reported were steatorrhea, "atypical" retinitis pigmentosa, progressive neurological deficits, and "thorny" red cells. The most complete case reports to date are those of Meir, Schwartz, and Boshes (3) and Rey (4), who well review the clinical aspects. The erythrocytes of affected individuals have char- acteristic, spikelike excrescences, a normal or de- creased life-span, probably normal osmotic fragil- ity, normal acid fragility, increased susceptibility to mechanical trauma, and an increased rate of destruction in lysolecithin hemolysis tests (2-5).