Furuta Natsumi, Tashiro Yuichi, Ikeda Masaki, Fujita Yukio, Okamoto Koichi
Department of Neurology, Gunma University Graduate School of Medicine.
Rinsho Shinkeigaku. 2012;52(3):186-9. doi: 10.5692/clinicalneurol.52.186.
This report deals with a 46-year-old male with Waldenström's macroglobulinemia (WM), who developed POEMS syndrome four years after diagnosis. The patient was diagnosed with WM, based on the presence of IgM-κ type monoclonal (M) protein and infiltration of lymphoplasmacytic cells identified in bone marrow aspirates. Four years later, the patient presented with progressive weakness and paresthesia of the limb extremities, and he was admitted to our hospital. Physical and neurological examination on admission revealed polyneuropathy, hepatosplenomegaly, hypothyroidism, IgM-κ M protein, leg edema, and cutaneous hyperpigmentation. He fulfilled the diagnostic criteria for POEMS syndrome. Laboratory tests showed normocytic normochromic anemia, elevated erythrocyte sedimentation rate, and increased levels of soluble IL-2 receptor, IL-6 and plasma vascular endothelial growth factor (VEGF). He was started on lenalidomide. After therapy, the leg edema and limb dysesthesia improved, and the VEGF level decreased from 608 pg/ml to 380 pg/ml. This is a very rare case of POEMS syndrome associated with WM, and is the first case treated with lenalidomide in Japan. VEGF presumably producted WM may be associated with development of POEMS syndrome.
本报告涉及一名46岁的男性，患有华氏巨球蛋白血症（WM），在诊断后四年出现POEMS综合征。该患者基于骨髓穿刺液中存在IgM-κ型单克隆（M）蛋白以及淋巴浆细胞浸润而被诊断为WM。四年后，患者出现进行性肢体无力和感觉异常，遂入住我院。入院时的体格检查和神经学检查发现有多发性神经病、肝脾肿大、甲状腺功能减退、IgM-κ M蛋白、腿部水肿和皮肤色素沉着。他符合POEMS综合征的诊断标准。实验室检查显示正细胞正色素性贫血、红细胞沉降率升高以及可溶性白细胞介素-2受体、白细胞介素-6和血浆血管内皮生长因子（VEGF）水平升高。他开始使用来那度胺治疗。治疗后，腿部水肿和肢体感觉异常改善，VEGF水平从608 pg/ml降至380 pg/ml。这是一例与WM相关的非常罕见的POEMS综合征病例，也是日本首例接受来那度胺治疗的病例。推测由WM产生的VEGF可能与POEMS综合征的发生有关。
