关键词: Waldenström macroglobulinemia monoclonal gammopathy multiple myeloma optic neuropathy

来  源:   DOI:10.3205/oc000240   PDF(Pubmed)

Abstract:
UNASSIGNED: To report a case of bilateral reversible optic neuropathy as the first sign of Waldenström macroglobulinemia (WM).
UNASSIGNED: Observational case report.
UNASSIGNED: A 52-year-old man had a sudden loss of vision in the left eye. Examinations revealed the presence of a serum monoclonal immunoglobulin (IgM kappa) in the serum. Even after a session of steroid pulse therapy, optic neuropathy became bilateral and then resolved almost completely after 4 months. The condition progressed to WM with multiorgan lesions years later. There was no evidence of optic neuropathy recurrence. The literature revealed two cases of monoclonal gammopathy (MG): a 64-year-old man with multiple myeloma (MM) with IgA lambda and a 51-year-old man with MM with IgG kappa. These cases have similar conditions: 1) visual reduction as an initial symptom of MG, 2) bilateral involvement, 3) no sign of central nervous system (CNS) infiltration shown by normal brain magnetic resonance images, and 4) recovery to a visual acuity of ≥1.0 bilaterally with no reoccurrence. The excessive Igs or B-cell hyperactivity may activate an autoimmune mechanism that reversibly interferes with the bilateral optic nerves.
UNASSIGNED: Bilateral optic neuropathy was the initial symptom of WM. There was no evidence of CNS infiltration; it recovered and then did not reoccur. The pathogenesis remained unknown, but two cases of MG were reported in the literature with remarkably similar conditions.
摘要:
报告一例以Waldenström巨球蛋白血症(WM)为首发征象的双侧可逆性视神经病变。
观察性病例报告。
一名52岁的男子左眼视力突然丧失。检查显示血清中存在血清单克隆免疫球蛋白(IgMκ)。即使在一次类固醇脉冲治疗之后,视神经病变发展为双侧,4个月后几乎完全缓解.多年后,病情发展为WM,并伴有多器官病变。没有视神经病变复发的证据。文献揭示了两例单克隆丙种球蛋白病(MG):一名64岁的患有IgAλ的多发性骨髓瘤(MM)的男性和一名51岁的患有IgGκ的MM的男性。这些病例具有相似的条件:1)视力下降作为MG的初始症状,2)双边参与,3)正常脑磁共振图像显示无中枢神经系统(CNS)浸润的迹象,和4)双侧视力恢复≥1.0,无复发。过度的Ig或B细胞过度活跃可能会激活一种可逆地干扰双侧视神经的自身免疫机制。
双侧视神经病变是WM的初始症状。没有中枢神经系统浸润的证据;它恢复了,然后没有复发。发病机制尚不清楚,但是文献中报道了2例MG,其情况非常相似。
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