Fleischer A S, Harber L C, Cook J S, Baer R L
Department of Dermatology, New York University, New York, NY, USA.
J Invest Dermatol. 1966 May;46(5):505-9. doi: 10.1038/jid.1966.76.
Four photosensitive patients with EPP showed markedly elevated RBC protoporphyrin levels. In vitro exposure of. a monolayer of their RBC in buffered saline to 3 x 10(6) ergs/mm2 of 4000 Å irradiation (= 45 minutes under a bank of fluorescent black lights) produced complete hemolysis within 24 hours; normal RBC showed negligible hemolysis after similar exposure. All of the cellular potassium was released from the patients' cells before the onset of significant hemolysis. Hemolysis was largely prevented by suspending the patients' cells in a 1:1 mixture of isotonic buffered NaCl and isotonic sucrose. These findings suggest that the initial photochemical lesion is of a "small hole" nature, creating channels through which small ions (but not sucrose) can diffuse. Since the effect of the intracellular colloid is offset by addition of sucrose to the external medium, the lesions appear to be less than 10 Å in diameter.
4例红细胞生成性原卟啉病（EPP）光敏患者的红细胞原卟啉水平显著升高。在缓冲盐水中，将他们的单层红细胞暴露于3×10⁶尔格/平方毫米的4000埃辐射下（=在一排荧光黑光灯下照射45分钟），24小时内出现完全溶血；正常红细胞在类似照射后溶血可忽略不计。在明显溶血发生之前，患者细胞内的所有钾离子都已释放。将患者的细胞悬浮于等渗缓冲氯化钠和等渗蔗糖的1:1混合物中，可在很大程度上防止溶血。这些发现表明，最初的光化学损伤具有“小孔”性质，形成了小离子（但不是蔗糖）能够扩散通过的通道。由于向外部介质中添加蔗糖可抵消细胞内胶体的作用，这些损伤的直径似乎小于10埃。
