PDF(Pubmed)
Abstract：
This study presents two cases of type II mixed cryoglobulinemia. One case is essential, while the other is presumably associated with hepatitis B virus (HBV) infection. Both patients tested positive for monoclonal IgMκ, but negative for MyD88 mutation. They showed resistance to rituximab combined with a glucosteroid regimen, but responded positively to BTK inhibitors. These cases highlight the remarkable effectiveness of BTK inhibitors in treating refractory type II cryoglobulinemia without MyD88 mutation. The first patient achieved rapid complete remission of nephrotic syndrome within one month of starting ibrutinib, along with a significant reduction in cryoglobulin levels and abnormal clonal cells. The second patient had a rapid disappearance of rash within three days and accelerated wound healing within one week of initiating orelabrutinib, accompanied by a reduction in C-reactive protein. However, there was no reduction in cryoglobulin levels during the 12-month follow-up. These findings suggest varied mechanisms of action of BTK inhibitors in type II cryoglobulinemia through different mechanisms.
摘要：
本研究提出了2例II型混合型冷球蛋白血症。一个案例是必不可少的，而另一种可能与乙型肝炎病毒（HBV）感染有关。两名患者的单克隆IgMκ检测呈阳性，但MyD88突变阴性.他们对利妥昔单抗联合糖皮质激素方案表现出耐药性，但对BTK抑制剂反应积极。这些病例突出了BTK抑制剂在治疗无MyD88突变的难治性II型冷球蛋白血症中的显着有效性。首例患者在开始伊布替尼后一个月内实现肾病综合征的快速完全缓解，随着冷球蛋白水平和异常克隆细胞的显着降低。第二名患者在开始奥列拉布替尼后三天内皮疹迅速消失，伤口愈合加速，伴随着C反应蛋白的减少。然而,12个月随访期间冷球蛋白水平没有降低.这些发现表明BTK抑制剂通过不同的机制在II型冷球蛋白血症中的不同作用机制。
