Garceau C, Leblond P F, Lyonnais J, Beaupré A
Nouv Rev Fr Hematol (1978). 1979 Jan 30;20(4):585-98.
In recent years, the presence of red cell morphological abnormalities in patients with Muscular Dystrophy has made the object of numerous, often contradictory reports. A possible source of such confusion may lie in the fact that human erythrocytes are extremely sensitive to morphologic transformations resulting from various manipulations or environmental conditions in vitro. We have examined the morphology and deformability of erythrocytes from 7 patients with Duchenne and 9 patients with Steinert (myotonic) Muscular Dystrophy. To avoid preparation artifacts, fresh, unwashed red cells suspended in their own plasma were examined under phase contrast microscopy for the presence of either echinocytes and stomatocytes. Deformability was measured by filtration of dilute cell suspensions at constant flow rate through nucleopore membranes (nominal pore diameter = 3 micrometer). No significant difference was found between the patients' cells and those of 22 healthy volunteer controls. We conclude that previously reported abnormalities may have been the result of preparation artifacts. It appears possible, however, that erythrocytes from Muscular Dystrophy patients may be more sensitive than normal ones to certain stimuli originating from red cell manipulations in vitro.
近年来，肌营养不良患者红细胞形态异常的情况成为众多报道的对象，这些报道往往相互矛盾。造成这种混淆的一个可能原因在于，人体红细胞对体外各种操作或环境条件导致的形态转变极为敏感。我们检查了7例杜氏肌营养不良患者和9例斯坦纳特（强直性）肌营养不良患者的红细胞形态和变形能力。为避免制备假象，将悬浮于自身血浆中的新鲜、未洗涤红细胞置于相差显微镜下检查是否存在棘形红细胞和口形红细胞。通过以恒定流速将稀释的细胞悬液过滤通过核孔膜（标称孔径 = 3微米）来测量变形能力。未发现患者的细胞与22名健康志愿者对照的细胞之间存在显著差异。我们得出结论，先前报道的异常情况可能是制备假象导致的。然而，肌营养不良患者的红细胞似乎有可能比正常红细胞对体外红细胞操作产生的某些刺激更为敏感。
