原发性巨球蛋白血症为一少见的免疫球蛋白异常疾患,迄今欧美报道仅400余例,日本报道80余例,国内自1973年以来所见文献报道有25例,我科近遇2例。本症发病缓慢,临床表现多样,早期诊断较困难,本文结合我科2例并复习国内所报道25例的有关资料,供临床诊断上的参考。 Primary macroglobulinemia is a rare disease of immunoglobulin abnormalities, so far only reported in Europe and America in more than 400 cases, more than 80 cases reported in Japan, the domestic literature reported since 1973, there were 25 cases, our department has been nearly 2 example. The onset of this disease is slow, diverse clinical manifestations, early diagnosis is more difficult, this article combined with 2 cases in our department and review the 25 cases reported in the domestic data for clinical diagnosis on the reference.