Nielsen H E, Lundh S, Jacobsen S V, Høiby N
Acta Paediatr Scand. 1978 Jul;67(4):443-7. doi: 10.1111/j.1651-2227.1978.tb16351.x.
Four patients are presented aged 14 to 20 years with cystic fibrosis and recurrent purpura of the legs. They have polyclonal increase of Ig but no intermediate complexes demonstrable by ultracentrifugation. The 4 patients differ from other patients with cystic fibrosis by a rapid deterioration of the clinical condition after the establishment of permanent pulmonary infection, and also by their proneness to Haemophilus influenzae infections. The clinical and laboratory findings are compatible with the diagnosis of Waldenström's hypergammaglobulinemic purpura. The heterogeneity of this syndrome is discussed.
本文报告了4例年龄在14至20岁之间的囊性纤维化患者，他们反复出现腿部紫癜。这些患者的免疫球蛋白呈多克隆性增加，但超速离心法未发现中间复合物。这4例患者与其他囊性纤维化患者不同，他们在发生永久性肺部感染后临床状况迅速恶化，且易感染流感嗜血杆菌。临床和实验室检查结果符合瓦尔登斯特伦高丙种球蛋白血症性紫癜的诊断。文中讨论了该综合征的异质性。
