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华氏巨球蛋白血症:45例病例回顾

Waldenström's macroglobulinemia: review of 45 cases.

作者信息

Krajny M, Pruzanski W

出版信息

Can Med Assoc J. 1976 May 22;114(10):899-900, 902, 905.

Abstract

Of 168 patients with monoclonal IgM in the serum 45 (27%) had Waldenström's macroglobulinemia. The mean age at diagnosis was 66 years. Generalized weakness, fatigue and bleeding manifestations were the usual chief complaints. Lymphadenopathy and hepatosplenomegaly were frequent. Moderate or severe anemia was noted in 29 patients, 9 had abnormal liver function, 8 had cold agglutinemia and 7 had cryoglobulinemia. Two patients had false-positive VDRL tests. The serum concentration of IgM usually exceeded 1000 mg/dl. Comparison of IgM values obtained by immunoquantitation and electrophoresis showed two types of discrepancy: readings were too high by immunoquantitation when IgM of low molecular weight was present, and readings were too low when, probably, IgM/IgG complexes were present or the serum was hyperviscous. Bence Jones protein was detected in 71% of the urine samples tested but the concentration was usually low, exceeding 200 mg/24 h in only nine instances. The mean survival time from the time of diagnosis for the 24 patients who died was 49.5 months and for the patients who are still alive, 43 months. Survival seemed to be related to the presence of azotemia, hypoalbuminemia and abnormal liver function.

摘要

在168例血清中存在单克隆IgM的患者中,45例(27%)患有华氏巨球蛋白血症。诊断时的平均年龄为66岁。常见的主要症状为全身无力、疲劳和出血表现。淋巴结病和肝脾肿大很常见。29例患者出现中度或重度贫血,9例肝功能异常,8例有冷凝集素血症,7例有冷球蛋白血症。2例患者VDRL试验呈假阳性。IgM血清浓度通常超过1000mg/dl。通过免疫定量和电泳获得的IgM值比较显示出两种差异类型:当存在低分子量IgM时,免疫定量读数过高;当可能存在IgM/IgG复合物或血清高粘滞时,读数过低。在71%的检测尿样中检测到本周蛋白,但浓度通常较低,仅9例超过200mg/24h。24例死亡患者从诊断时起的平均生存时间为49.5个月,仍存活患者的平均生存时间为43个月。生存似乎与氮质血症、低白蛋白血症和肝功能异常的存在有关。

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本文引用的文献

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Calculation of survival rates for cancer.癌症生存率的计算
Proc Staff Meet Mayo Clin. 1950 May 24;25(11):270-86.
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Ann Intern Med. 1963 May;58:789-800. doi: 10.7326/0003-4819-58-5-789.
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The histopathology of the macroglobulinemia of Waldenström.华氏巨球蛋白血症的组织病理学
J Natl Cancer Inst. 1959 May;22(5):887-917. doi: 10.1093/jnci/22.5.887.
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Clinical and haematological aspects of macroglobulinaemia Waldenström.华氏巨球蛋白血症的临床和血液学方面
Acta Med Scand. 1959 Apr 15;163(5):349-66. doi: 10.1111/j.0954-6820.1959.tb10414.x.
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An IgM pyroglobulin associated with lymphosarcoma.一种与淋巴肉瘤相关的IgM热球蛋白。
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