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多发性骨髓瘤的诊断标准。

Diagnostic criteria of multiple myeloma.

作者信息

Kyle R A

机构信息

Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minnesota.

出版信息

Hematol Oncol Clin North Am. 1992 Apr;6(2):347-58.

PMID:1582977
Abstract

Immunoelectrophoresis or immunofixation is necessary for the identification of a monoclonal protein. During 1990 at the Mayo Clinic, 787 patients were found to have a monoclonal gammopathy. IgG accounted for 61% of the cases, followed by IgM (18%), IgA (11%), Bence Jones proteinemia (6%), biclonal gammopathy (3.5%), and IgD (0.5%). Monoclonal gammopathy of undetermined significance accounted for approximately two thirds of patients. This denotes the presence of a monoclonal protein in persons without evidence of multiple myeloma, macroglobulinemia, amyloidosis, or other related diseases. During long-term follow-up of patients with monoclonal gammopathy of undetermined significance, we found that one fourth developed multiple myeloma or related disorders. The interval from recognition of the monoclonal gammopathy to the diagnosis of multiple myeloma ranged from 2 to 29 years (median, 10 years). Waldenström's macroglobulinemia developed in seven patients 4 to 20 years (median, 8.5 years) after recognition of the monoclonal protein. Systemic amyloidosis (AL) was found in eight patients 6 to 19 years (median, 9 years) after the diagnosis of a serum monoclonal protein. Five patients developed a malignant lymphoproliferative process 6 to 22 years (median, 10.5 years) after recognition of a monoclonal protein. Minimal criteria for the diagnosis of multiple myeloma include the presence of at least 10% abnormal plasma cells in the bone marrow or histologic proof of a plasmacytoma, the usual clinical features of multiple myeloma, and at least one of the following abnormalities: monoclonal serum protein (usually greater than 3 g/dL), monoclonal protein in the urine, or osteolytic lesions. No single technique differentiates benign from malignant plasma cell proliferation. The most dependable means is serial measurement of the monoclonal protein in the serum and urine and periodic reevaluation of pertinent clinical and laboratory features to determine whether multiple myeloma, systemic amyloidosis, macroglobulinemia, or other lymphoplasma cell proliferative disease has developed.

摘要

免疫电泳或免疫固定对于单克隆蛋白的鉴定是必要的。1990年在梅奥诊所,发现787例患者患有单克隆丙种球蛋白病。IgG占病例的61%,其次是IgM(18%)、IgA(11%)、本周氏蛋白血症(6%)、双克隆丙种球蛋白病(3.5%)和IgD(0.5%)。意义未明的单克隆丙种球蛋白病约占患者的三分之二。这表明在无多发性骨髓瘤、巨球蛋白血症、淀粉样变性或其他相关疾病证据的人群中存在单克隆蛋白。在对意义未明的单克隆丙种球蛋白病患者进行长期随访期间,我们发现四分之一的患者发展为多发性骨髓瘤或相关疾病。从识别单克隆丙种球蛋白病到诊断多发性骨髓瘤的间隔时间为2至29年(中位数为10年)。7例患者在识别单克隆蛋白后4至20年(中位数为8.5年)发生了华氏巨球蛋白血症。在诊断血清单克隆蛋白后6至19年(中位数为9年),8例患者发现了系统性淀粉样变性(AL)。5例患者在识别单克隆蛋白后6至22年(中位数为10.5年)发生了恶性淋巴细胞增殖性疾病。多发性骨髓瘤的诊断最低标准包括骨髓中至少10%的异常浆细胞或浆细胞瘤的组织学证据、多发性骨髓瘤的常见临床特征以及以下至少一项异常:单克隆血清蛋白(通常大于3 g/dL)、尿中单克隆蛋白或溶骨性病变。没有单一技术能区分良性和恶性浆细胞增殖。最可靠的方法是对血清和尿液中的单克隆蛋白进行系列检测,并定期重新评估相关临床和实验室特征,以确定是否已发展为多发性骨髓瘤、系统性淀粉样变性、巨球蛋白血症或其他淋巴浆细胞增殖性疾病。

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