文献简介

出版社:Clin Rheumatol

作  者:Niklas T. Baerlecken & Reinhold E. Schmidt

编  号:10.1007/s10067-010-1412-8

关键字:Cryoglobulinemia, Leukocytoclastic vasculitis ,Rituximab

年  份:2013   点击量:647

文献摘要

We report about a 39-year-old female patient with severe essential mixed cryoglobulinemia of type III with leukocytoclastic vasculitis. The patient was admitted into our hospital with mesenteric lymphangiitis, which caused enteral perforation, sepsis, and pneumonia. Cryoglobulins, cryocrit, Ig-titers, and biopsy were positive for mixed cryoglobulinemia type III. We detected no signs of hepatitis C, B, or any other infectious disease. At first, disease activity could be kept under control with high doses of glucocorticoids and multiple cyclophosphamide pulses. However, after therapy with three pulses of rituximab, steroids were stopped, and the patient has not presented any symptoms for 2 years. Therefore, we suggest that rituximab affected her disease rapidly and effectively. In conclusion, rituximab is an alternative therapy for mixed cryoglobulinemia of type III with leukocytoclastic vasculitis.

本文报道一例患有严重的伴有白细胞破碎性血管炎的Ⅲ型原发性混合性冷球蛋白血症的39岁女性患者病例。患者入院时患有肠系膜淋巴管炎,并引发了肠穿孔、败血症和肺炎。Ⅲ型混合性冷球蛋白血症中冷球蛋白、冷沉[淀]比容、免疫球蛋白滴度,以及活检均呈阳性。未检测到丙型肝炎、乙型肝炎或其他感染性疾病的迹象。起初,通过使用高剂量的糖皮质激素和多倍剂量环磷酰胺,可控制疾病活动度。然而,应用三倍剂量的利妥昔单抗治疗后、停止应用糖皮质激素,患者2年内未出现任何症状。因此,本文认为,利妥昔单抗可以迅速并有效的缓解了患者症状。总之,利妥昔单抗是治疗伴有白细胞破碎性血管炎的Ⅲ型混合性冷球蛋白血症的一种替代疗法。