关键词: Waldenström disease chronic hepatitis C cryoglobulinemia double-filtration plasmapheresis membranoproliferative glomerulonephritis rituximab

Mesh : Humans Male Adult Middle Aged Aged Cryoglobulinemia / therapy Plasmapheresis Blood Component Removal Vasculitis / therapy Hepacivirus

来  源:   DOI:10.1111/1744-9987.13885

Abstract:
BACKGROUND: Cryoglobulinemia is defined as the presence of an abnormal immunoglobulin that may be responsible for vasculitis of small-caliber vessels. Apheresis can be used in order to temporarily eliminate circulating cryoglobulins. The aim of this study was to assess the effectiveness of apheresis (double-filtration plasmapheresis-DFPP-) in symptomatic and/or severe cryoglobulinemias.
METHODS: Four male patients presenting cryoglobulinemic vasculitis and who received DFPP sessions were included.
RESULTS: Their mean age was 57 ± 15 years. One patient had hepatitis-C virus (HCV)-related cryoglobulinemia and the other three patients were carriers of an IgM Kappa monoclonal gammopathy. Mean duration of follow-up was 15 ± 2 months. DFPP allowed healing of ulcerative skin lesions in the first patient and remission of nephrotic syndrome in the other patients after a median of 6(5-10) sessions.
CONCLUSIONS: DFPP can be used safely in cryoglobulinemic-vasculitis and can be considered early to achieve a faster and sustained clinical-biological response.
摘要:
背景:冷球蛋白血症被定义为存在异常的免疫球蛋白,这可能是小口径血管血管炎的原因。可以使用单采术以暂时消除循环冷球蛋白。这项研究的目的是评估单采(双重过滤血浆置换-DFPP-)在有症状和/或严重冷球蛋白血症中的有效性。
方法:纳入了4名出现冷球蛋白血症性血管炎并接受DFPP治疗的男性患者。
结果:他们的平均年龄为57±15岁。一名患者患有丙型肝炎病毒(HCV)相关的冷球蛋白血症,其他三名患者是IgMκ单克隆丙种球蛋白病的携带者。平均随访时间为15±2个月。在中位数为6(5-10)次之后,DFPP允许第一例患者的溃疡性皮肤病变愈合,其他患者的肾病综合征缓解。
结论:DFPP可以安全地用于冷球蛋白性血管炎,并且可以早期考虑实现更快和持续的临床生物学反应。
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