Budmiger H, Rhyner K, Siegenthaler-Zuber G, Bollinger A
Departement für Innere Medizin, Universitätsspital Zürich.
Schweiz Med Wochenschr. 1988 Jan 16;118(2):52-7.
Clinical features, diagnostic procedure, therapy, course of the disease and prognosis in 6 patients with severe idiopathic chronic cold agglutinin disease are described. In 5 patients the main complaint was cold mediated acrocyanosis. The cold agglutinin in all patients was of anti-I type and belonged to IgM immunoglobulin. Keeping warm provided symptomatic relief and the hemolysis decreased to a milder form. Treatment with glucocorticoids alone failed in two patients but succeeded in combination with chlorambucil or cyclophosphamid. One patient developed a lymphoproliferative disorder 11 years after diagnosis of idiopathic chronic cold agglutinin disease.
本文描述了6例严重特发性慢性冷凝集素病患者的临床特征、诊断方法、治疗、病程及预后。5例患者的主要症状为寒冷介导的手足发绀。所有患者的冷凝集素均为抗I型，属于IgM免疫球蛋白。保暖可缓解症状，溶血减轻至较轻形式。单独使用糖皮质激素治疗，2例患者无效，但联合苯丁酸氮芥或环磷酰胺治疗成功。1例患者在诊断为特发性慢性冷凝集素病11年后发生了淋巴增殖性疾病。
