Chronic lymphocytic leukemia (CLL) can trigger autoimmune phenomena, with immune thrombocytopenia (ITP) the most common presentation. Upon cessation of CLL therapy, including ibrutinib, autoimmune flares can occur. In a 68-year-old man with CLL, ibrutinib was held for 2 weeks prior to elective shoulder surgery. Eleven days after stopping therapy, he presented with a purpuric rash on his right hip, buttock, and lower extremities. He experienced two episodes of seizure activity while hospitalized. MRI brain demonstrated patchy areas of altered signal involving deep white matter and sub-cortical white matter structures concerning for cerebral vasculitis. Although there was no evidence of hemolysis, serum cold agglutinin titer was elevated at > 1:512 and cryoglobulin levels were positive at 36%. He was diagnosed with type I cryoglobulinemia and treated with rituximab, plasmapheresis, methylprednisolone, and ibrutinib was restarted. This regimen resolved his symptoms. A rare complication of CLL is the production of cryoglobulins, which can present at initial diagnosis or in relapsed disease. Our case demonstrates that the cessation of ibrutinib therapy, even for a short time, can precipitate complications. To our knowledge, we report the first case of a patient with well-controlled CLL who rapidly developed cryoglobulinemic vasculitis after stopping ibrutinib therapy.

中文翻译：

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中断依鲁替尼治疗慢性淋巴细胞白血病 (CLL) 的冷球蛋白血症性血管炎。

慢性淋巴细胞白血病 (CLL) 可引发自身免疫现象，其中免疫性血小板减少症 (ITP) 是最常见的表现。在停止包括依鲁替尼在内的 CLL 治疗后，可能会发生自身免疫性发作。在一名患有 CLL 的 68 岁男性中，在择期肩部手术前，依鲁替尼被停药 2 周。停止治疗 11 天后，他的右臀部、臀部和下肢出现紫癜性皮疹。他在住院期间经历了两次癫痫发作。MRI 大脑显示涉及脑血管炎的深部白质和皮层下白质结构的斑片状信号改变区域。虽然没有溶血的证据，但血清冷凝集素滴度升高 > 1:512，冷球蛋白水平为阳性，为 36%。他被诊断为 I 型冷球蛋白血症，并接受了利妥昔单抗、血浆置换、甲基强的松龙和依鲁替尼治疗。这个方案解决了他的症状。CLL 的一个罕见并发症是冷球蛋白的产生，它可以在初始诊断时或在疾病复发时出现。我们的案例表明，停止依鲁替尼治疗，即使是很短的时间，也会引发并发症。据我们所知，我们报告了首例控制良好的 CLL 患者，该患者在停止依鲁替尼治疗后迅速发展为冷球蛋白血症性血管炎。我们的案例表明，停止依鲁替尼治疗，即使是很短的时间，也会引发并发症。据我们所知，我们报告了首例控制良好的 CLL 患者，该患者在停止依鲁替尼治疗后迅速发展为冷球蛋白血症性血管炎。我们的案例表明，停止依鲁替尼治疗，即使是很短的时间，也会引发并发症。据我们所知，我们报告了首例控制良好的 CLL 患者，该患者在停止依鲁替尼治疗后迅速发展为冷球蛋白血症性血管炎。