Shigemi H, Murakami S, Oku N, Itoh K, Fujita N, Nakanishi S, Shimazaki C, Inoue D, Takahashi H, Nakagawa M
Second Department of Medicine, Kyoto Prefectural University of Medicine.
Rinsho Ketsueki. 1990 Feb;31(2):209-13.
A 43-year-old man was admitted to our hospital because of legs edema and periorbital edema in Dec. 1983. Laboratory findings showed massive proteinuria (3.7 g/day), Bence Jones protein (BJP) in urine, and hypoproteinemia. Peripheral blood examinations were normal and a bone marrow aspiration showed hypocellularity with slight increase of monocytes and plasma cells. Serum immunoelectrophoresis showed two M-components (IgG kappa, IgA lambda). Serum IgG was 1,690 mg/dl, IgA 379 mg/dl and IgM 160 mg/dl. No remarkable findings were obtained in bone survey, Ga-scintigraphy and rectal biopsy, and a diagnosis of diclonal gammopathy with nephrotic syndrome was made. In Aug. 1986, serum IgA started to increase rapidly with concomitant decrease IgG. He died of pneumonia due to pancytopenia in Dec. 1986, when serum IgG was 450 mg/dl, IgA 1,014 mg/dl, and IgM less than 39 mg/dl. Immunoelectrophoresis showed two M-components (IgG kappa, IgA lambda) in serum and BEP (kappa, lambda), IgG (kappa) and IgA (lambda) in urine. An autopsy showed massive infiltration of myeloma cells which were positive for lambda light chain in bone marrow, suggesting a development of myeloma from a diclonal gammopathy in about 3 years.
1983年12月，一名43岁男性因腿部水肿和眶周水肿入院。实验室检查发现大量蛋白尿（3.7克/天）、尿本周蛋白（BJP）和低蛋白血症。外周血检查正常，骨髓穿刺显示细胞减少，单核细胞和浆细胞略有增加。血清免疫电泳显示两个M成分（IgG κ、IgA λ）。血清IgG为1690毫克/分升，IgA为379毫克/分升，IgM为160毫克/分升。骨骼检查、镓闪烁扫描和直肠活检均未发现明显异常，诊断为双克隆丙种球蛋白病伴肾病综合征。1986年8月，血清IgA开始迅速升高，同时IgG降低。1986年12月，他因全血细胞减少死于肺炎，当时血清IgG为450毫克/分升，IgA为1014毫克/分升，IgM低于39毫克/分升。免疫电泳显示血清中有两个M成分（IgG κ、IgA λ），尿中有BEP（κ、λ）、IgG（κ）和IgA（λ）。尸检显示骨髓瘤细胞大量浸润，骨髓中λ轻链呈阳性，提示约3年内双克隆丙种球蛋白病发展为骨髓瘤。
