Acquired Acanthocytosis and Myelophthisis in a Case of Eales's Disease.

ACANTHOCYTOSIS, a congenital anomaly of the erythrocyte surface membranes with thorny protuberances due to a defect of beta-lipoproteins (Singer, Fisher, and Perlstein, 1952; Jampel and Falls, 1958; Mier, Schwartz, and Boshes, 1960; Druez, Lamy, Frezal, Polonovski, and Rey, 1961) has been described in association with atypical retinitis pigmentosa (Bassen and Kornzweig, 1950) and has also been found in association with hereditary vitreo-retinal degeneration (degeneratio hyaloideoretinalis: Wagner, 1938; Kahain, Kahan, Benko, and Mindszenti, 1963). One of the most striking features of the latter is hereditary retinoschisis (Mann and Macrae, 1938; Juler, 1947; Sorsby, Klein, Gann, and Siggins, 1951; Gieser and Falls, 1961) originating, as suggested by Scorciarini-Coppola, Orlando, and d'Antuono (1958), from vaso-obstruction. A case of Eales's disease with acquired acanthocytosis, a very low level of non-esterified cholesterol, and almost complete absence of haematopoiesis in the sternal bone marrow, is described below. Donner (1953) stated that the basic phenomenon of retinal periphlebitis is also an obstruction of vessels by erythrocytes. Reduction or complete disappearance of digitoninprecipitable (non-esterified) cholesterol may also be obtained by the injection of endotoxins (Meier and Schuler, 1957). This change in lipids was found to induce transient acanthocytosis. The biochemical, morphological, and haematological aspects of congenital, acquired, and induced acanthocytosis have much in common, and may have a role in the vascular obstructions of hereditary retinoschisis and retinal periphlebitis, clinical entities of different aetiology but similar pathogenesis.