Kyle R A
Division of Haematology and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA.
Baillieres Clin Haematol. 1995 Dec;8(4):761-81. doi: 10.1016/s0950-3536(05)80258-6.
MGUS is characterized by a serum M-protein concentration of less than 30 milligrams (3 g/dl), fewer than 10% plasma cells in the bone marrow, no or only small amounts of M-protein (Bence Jones protein) in the urine, the absence of lytic lesions, anaemia, hypercalcaemia and renal insufficiency, and most importantly, stability of the M-protein and failure of the development of additional abnormalities. Electrophoresis on agarose, followed by immunoelectrophoresis or immunofixation for the identification of the type of M-protein, is recommended. In 1994, 971 patients at the Mayo Clinic were found with a serum M-protein. The most frequent diagnosis was MGUS, which occurred in 52% of patients. MGUS is found in approximately 3% of people older than 70 years and in at least 1% of those aged over 50. The incidence of monoclonal gammopathies increases with advancing age and is higher in African-Americans than in Caucasians. Two hundred and forty-one patients from the Mayo Clinic with a monoclonal gammopathy but no evidence of MM, macroglobulinaemia, amyloidosis, lymphoma or related disorders were followed for 24-38 years. In 62 patients (26%), multiple myeloma, macroglobulinaemia, amyloidosis or a malignant lymphoproliferative disorder developed (the actuarial rate of development of serious disease at 10 years was 16%; at 20 years, 33%; and at 25 years, 40%). Thirty patients (12%) were alive and had a stable M-protein value. In 23 patients (10%), the serum M-protein level increased to 30 milligrams (3 g/dl) or more, but they did not require therapy for myeloma or related disorders. Fifty-two per cent of patients (126) died of unrelated diseases without the development of a malignant plasma cell lymphoproliferative disorder. The actual rate of development of serious disease was the same for those with IgG, IgA and IgM M-proteins. Differentiation of MGUS from myeloma or macroglobulinaemia is difficult. The M-protein value must be measured periodically and clinical evaluation carried out to determine whether or not serious disease has developed.
意义未明的单克隆丙种球蛋白病(MGUS)的特征为:血清M蛋白浓度低于30毫克(3g/dl),骨髓中浆细胞少于10%,尿中无M蛋白或仅有少量M蛋白(本周蛋白),无溶骨性损害、贫血、高钙血症及肾功能不全,最重要的是,M蛋白稳定且无其他异常情况出现。建议采用琼脂糖电泳,随后进行免疫电泳或免疫固定电泳以鉴定M蛋白类型。1994年,梅奥诊所发现971例患者有血清M蛋白。最常见的诊断是MGUS,占患者的52%。MGUS在70岁以上人群中约占3%,在50岁以上人群中至少占1%。单克隆丙种球蛋白病的发病率随年龄增长而增加,非裔美国人高于白种人。对梅奥诊所241例患有单克隆丙种球蛋白病但无多发性骨髓瘤、巨球蛋白血症、淀粉样变性、淋巴瘤或相关疾病证据的患者进行了24至38年的随访。62例患者(26%)发生了多发性骨髓瘤、巨球蛋白血症、淀粉样变性或恶性淋巴增殖性疾病(10年时严重疾病的实际发生率为16%;20年时为33%;25年时为40%)。30例患者(12%)存活且M蛋白值稳定。23例患者(10%)血清M蛋白水平升至30毫克(3g/dl)或更高,但无需接受骨髓瘤或相关疾病的治疗。52%的患者(126例)死于无关疾病,未发生恶性浆细胞淋巴增殖性疾病。IgG、IgA和IgM M蛋白患者发生严重疾病的实际发生率相同。MGUS与骨髓瘤或巨球蛋白血症的鉴别困难。必须定期检测M蛋白值并进行临床评估,以确定是否已发生严重疾病。