SAWADA Takeshi, NAJIMA Yuho, OHASHI Kazuteru, KATO Ikuma, MIYAZAWA Maho, NAKANO Mikako, KOBAYASHI Takeshi, YAMASHITA Takuya, AKIYAMA Hideki, SAKAMAKI Hisashi
Hematology Division, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital.
Rinsho Ketsueki. 2009 Nov;50(11):1635-40.
We herein describe a rare case of multiple myeloma with an aggressive clinical course and the unusual manifestation of multiple organ involvement by plasma cells. A 58-year-old man noted difficulty in walking due to progressive swelling of his left lower limb. CT scan revealed a huge mass in the inguinal region in addition to masses located on the head, and in the aero-digestive tract and spinal canal. The pathological diagnosis of plasmacytoma was made on biopsied specimens of these masses, while plasma cells did not increase (5.8%) in aspirated bone marrow obtained at the same time. Serum IgG level was 6,387 mg/dl and immunoelectrophoresis demonstrated monoclonal IgG-kappa in the serum. Chemotherapy with vincristine, adriamycin, dexamethasone, subsequent high-dose cyclophosphamide, and irradiation involving both thoracic vertebral canal and inguinal regions resulted in improvement of initial symptoms. However, the patient relapsed soon after; new lesions developed in various parts of the body, including the left thigh and body trunk. Salvage therapy including bortezomib was no longer effective, and he eventually died 10 months after the initial diagnosis. Autopsy revealed the diffuse involvement of plasma cells of multiple organs, including the liver, spleen, abdominal lymph node, and bone marrow in addition to the left leg.
我们在此描述一例罕见的多发性骨髓瘤病例，其临床病程侵袭性强，且浆细胞出现多器官受累的异常表现。一名58岁男性因左下肢进行性肿胀而行走困难。CT扫描显示腹股沟区有一个巨大肿块，此外头部、气道消化道和椎管内也有肿块。对这些肿块的活检标本进行病理诊断为浆细胞瘤，而同时采集的骨髓穿刺液中浆细胞未增多（5.8%）。血清IgG水平为6387mg/dl，免疫电泳显示血清中有单克隆IgG-κ。采用长春新碱、阿霉素、地塞米松化疗，随后给予大剂量环磷酰胺，并对胸椎椎管和腹股沟区进行放疗，初始症状有所改善。然而，患者很快复发；身体各部位出现新病灶，包括左大腿和躯干。包括硼替佐米在内的挽救治疗不再有效，他最终在初次诊断后10个月死亡。尸检显示除左腿外，多个器官包括肝脏、脾脏、腹部淋巴结和骨髓均有浆细胞弥漫性浸润。
