Rosse W F, Parker C J
Clin Haematol. 1985 Feb;14(1):105-25.
The analysis of the abnormality of the membrane in paroxysmal nocturnal haemoglobinuria is an exercise in complexity complexified--of Ossa atop Pelion. To understand it, we will need to understand fully the complexities of the complement system and the complexities of the structure of the membrane and the complexity of their concert interaction. Added to this are the complexities of growth and development of the haematopoietic cells--a problem we have not discussed at all despite its importance in the pathophysiology of PNH. It is little wonder that a disease which is uncommon but not rare continues to fascinate investigators from a wide variety of disciplines. If, as has been (falsely) said, more people studied the disease than had it, it would be because it presents questions of such fundamental interest. In unravelling its mysteries, many problems related to the normal functioning of cells, complement and a haematopoiesis have been and will continue to be solved.
阵发性夜间血红蛋白尿中膜异常的分析是一项复杂程度不断升级的工作——如同叠床架屋。要理解它,我们需要充分了解补体系统的复杂性、膜结构的复杂性以及它们协同相互作用的复杂性。此外,造血细胞生长和发育的复杂性也不容忽视——尽管这在阵发性夜间血红蛋白尿的病理生理学中至关重要,但我们根本未曾讨论过这个问题。毫不奇怪,这样一种虽不常见但也并非罕见的疾病,一直吸引着来自广泛学科领域的研究者。如果(错误地)说研究这种疾病的人比患这种病的人还多,那是因为它提出了如此具有根本意义的问题。在揭开其奥秘的过程中,许多与细胞、补体和造血正常功能相关的问题已经并将继续得到解决。