Djaldetti M
Department of Medicine "B", Hasharon Hospital, Petah-Tiqua, Israel.
CRC Crit Rev Clin Lab Sci. 1978;9(1):49-83. doi: 10.3109/10408367809150915.
The clinical and hematological features of a rare autoimmune disorder, PCH, are reviewed. Based on the case reports of 24 patients suffering from this disease, the presence of cold hemolysins in the sera of these patients as the main cause for the red cell destruction following exposure to cold is further discussed. However, recent electron microscope and biochemical studies suggest, in addition, that alterations of the red cells, and especially of their membranes, may play a role in the hemolytic process. The role of other factors, such as thermal range, specificity, and the immunological properties of the Donath-Landsteiner antibodies, as well as the role of the complement in the cold and warm phase of the Donath-Landsteiner reaction, is discussed. The differential diagnosis between PCH and the closely related CHD is outlined. While most of the reports deal with the mechanism of red cell destruction, relatively little attention is given to the treatment of the disease. It appears that the oldest remedy, i.e., warming of the patient and prevention of his exposure to cold, remains the best.
本文回顾了一种罕见的自身免疫性疾病——阵发性寒冷性血红蛋白尿（PCH）的临床和血液学特征。基于24例该疾病患者的病例报告，进一步讨论了这些患者血清中冷溶血素的存在是其红细胞在接触寒冷后被破坏的主要原因。然而，最近的电子显微镜和生化研究还表明，红细胞尤其是其细胞膜的改变可能在溶血过程中起作用。文中还讨论了其他因素的作用，如多纳-兰斯泰纳抗体的温度范围、特异性和免疫特性，以及补体在多纳-兰斯泰纳反应的冷相和温相中的作用。概述了PCH与密切相关的冷凝集素病（CHD）之间的鉴别诊断。虽然大多数报告关注红细胞破坏的机制，但对该疾病的治疗相对关注较少。看来最古老的治疗方法，即让患者保暖并防止其暴露于寒冷环境，仍然是最佳方法。
