Fujii H, Nakagawa H, Kanoh T
Third Department of Internal Medicine, Kyoto First Red Cross Hospital.
Rinsho Ketsueki. 1992 Nov;33(11):1708-13.
This report deals with an unusual case of primary macroglobulinemia with hypercalcemia, chronic renal failure and systemic amyloidosis. In May 1990, a 63-year-old male was transferred to our hospital because of hypercalcemia (13.5 mg/dl) and renal failure. Clinical examinations showed anemia, macroglossia, lymph node swellings and hepatomegaly. Laboratory findings included Bence-Jones (kappa type) proteinuria (0.8 g/day), a monoclonal gammopathy of the IgM-kappa type (2.8 g/dl), a proliferation of lymphoid cells in the peripheral blood (5%) and the bone marrow (59.6%), and lymphomatous involvement of an inguinal lymph node. Serum creatinine concentration was 8.5 mg/dl. The serum levels of parathormone and vitamin D3 metabolites were normal. The roentgenogram of bones showed a compression fracture of the lumbar spine and systemic osteoporosis. The treatment included eel calcitonin, prednisolone and the CHOP regimen, followed by hemodialysis and plasmapheresis. The serum level of IgM increased to 4.6 g/dl. The patient died three months later and postmortem examination demonstrated marked systemic amyloidosis.
本报告涉及一例罕见的原发性巨球蛋白血症病例，伴有高钙血症、慢性肾衰竭和系统性淀粉样变性。1990年5月，一名63岁男性因高钙血症（13.5mg/dl）和肾衰竭被转至我院。临床检查发现贫血、巨舌、淋巴结肿大和肝肿大。实验室检查结果包括本周氏蛋白（κ型）蛋白尿（0.8g/天）、IgM-κ型单克隆丙种球蛋白病（2.8g/dl）、外周血淋巴细胞增殖（5%）和骨髓淋巴细胞增殖（59.6%），以及腹股沟淋巴结淋巴瘤浸润。血清肌酐浓度为8.5mg/dl。甲状旁腺激素和维生素D3代谢产物的血清水平正常。骨骼X线片显示腰椎压缩性骨折和全身性骨质疏松。治疗包括鳗鱼降钙素、泼尼松龙和CHOP方案，随后进行血液透析和血浆置换。IgM血清水平升至4.6g/dl。患者三个月后死亡，尸检显示明显的系统性淀粉样变性。
