Department of Clinical Therapeutics, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
2 Department of Neurology, School of Medicine, National and Kapodistrian University of Athens, °"Attikon" General University Hospital, Athens, Greece.
In Vivo. 2024 Mar-Apr;38(2):954-957. doi: 10.21873/invivo.13526.
BACKGROUND/AIM: The presence of a monoclonal gammopathy of undetermined significance (MGUS) even in small amounts may trigger tissue damage through immunological or other mechanisms, irrespective of the potential for malignant transformation. The aim of the study was to present a case of monoclonal gammopathy of clinical significance with ocular manifestations and discuss relevant literature.
In our case, a patient presented with vision disturbances that was eventually attributed to the underlying IgM MGUS after extensive workup to exclude other potential etiologies. The patient showed a clinical response with the fixed-duration DRC (dexamethasone, rituximab, cyclophosphamide) regimen that persisted for at least 1.5 years. Herein, we present, in detail, the patient management and discuss the underlying pathophysiology of this rare entity with few available published data in this field.
A high level of clinical suspicion is necessary in order to detect the association between MGUS and a clinical sign or symptom that cannot be attributed elsewhere.
背景/目的：即使是少量的意义未明的单克隆丙种球蛋白血症（MGUS）也可能通过免疫或其他机制引发组织损伤，而不论其恶性转化的可能性如何。本研究的目的是报告一例具有眼部表现的临床意义单克隆丙种球蛋白血症病例，并讨论相关文献。
在我们的病例中，患者出现视力障碍，经过广泛的检查以排除其他潜在病因后，最终归因于潜在的 IgM MGUS。患者在固定疗程的 DRC（地塞米松、利妥昔单抗、环磷酰胺）方案治疗后表现出临床反应，至少持续了 1.5 年。在此，我们详细介绍了患者的治疗情况，并讨论了该罕见疾病的潜在病理生理学，该领域的可用数据很少。
为了发现 MGUS 与无法归因于其他原因的临床体征或症状之间的关联，需要高度的临床怀疑。
