小结
定义
病史和体格检查
关键诊断因素
- 年龄＞70 岁
- 男性
- 白人血统
其他诊断因素
- IgM 型意义未明的单克隆免疫球蛋白血症（MGUS）病史
- B 细胞淋巴瘤或骨髓瘤家族史
- WM 家族史（伴或不伴意义未明的单克隆免疫球蛋白血症 [monoclonal gammopathy of undetermined significance, MGUS] 和多发性骨髓瘤）
- 疲劳
- 厌食
- 感染
- 周围神经病
- 体重减轻
- 发热
- 雷诺现象
- 脾肿大
- 淋巴结肿大
- 肝肿大
- 紫癜
- 皮肤和/或黏膜出血
- 眼科症状
- 头痛
- 头晕和/或眩晕
- 血栓
危险因素
- IgM 型意义未明的单克隆丙种球蛋白病 (MGUS)
- B 淋巴细胞增生性疾病或多发性骨髓瘤家族史
- WM 家族史
- 丙型肝炎
诊断性检查
首要检查
- FBC 和分类计数
- 血红素检测（铁、维生素 B12 和叶酸）
- 全套肾功能检查
- 肝功能检查 (LFT)
- C 反应蛋白 (CRP)
- 高分辨率血清蛋白电泳 (Serum Protein Electrophoresis, SPEP) 与免疫固定
- 血清游离轻链
- 冷凝集素和冷球蛋白
- 乳酸脱氢酶
- β-2 微球蛋白
- 血清白蛋白
- 相对血清黏滞度
- 骨髓活检
- 胸部、腹部和盆腔 CT
需考虑的检查
- 淋巴结活组织检查
- 胸部、腹部和盆腔 18-F-脱氧葡萄糖 PET/CT 扫描
- 定量免疫球蛋白
- 抗髓鞘相关糖蛋白（MAG）抗体
- 抗硫脂 IgM 抗体
- 肌电图
- 脂肪垫活检
- 凝血酶原时间（Prothrombin time, PT）和活化部分凝血活酶时间（activated partial thromboplastin time, aPTT）
- 病毒血清学检查（乙型肝炎、丙型肝炎和 HIV）
- 基因突变测试
治疗流程
无症状
有症状，肿瘤负荷低
有症状，肿瘤负荷高
对含利妥昔单抗的初始治疗有应答的患者
复发或难治性疾病
撰稿人
作者
Guy Pratt, MD, FRCP, FRCPath
Honorary Consultant Haematologist
University Hospitals Birmingham NHS Foundation Trust
Professor of Haematology
Institute of Cancer and Genomic Sciences
College of Medical and Dental Sciences
University of Birmingham
Birmingham
UK
利益声明
GP has received honoraria for speaking, advisory board memberships, and travel support from Janssen, BMS-Celgene, Takeda, Binding Site Ltd, and Amgen. GP is an author of references cited within this topic.
鸣谢
Dr Guy Pratt wishes to gratefully acknowledge Dr Boris Kobrinsky and Dr Kenneth Hymes, the previous contributors to this topic.
利益声明
BK and KH declare that they have no competing interests.
同行评议者
Shaji Kumar, MD
Consultant
Department of Hematology
Mayo Clinic
Rochester
MN
利益声明
SK declares that he has no competing interests.
Madhav Dhodapkar, MD
Professor of Medicine
Chief, Section of Hematology
Department of Internal Medicine
Yale University School of Medicine
New Haven
CT
利益声明
MD declares that he has no competing interests.
Xavier Leleu, MD, PhD
Instructor in Hematology
Department of Hematology
Hopital Huriez CHRU
Lille
France
利益声明
XL has received lecture fees and research funding from Janssen-Cilag, Celgene, Chugai, Amgen, Novartis, Mundipharma, and Roche. XL is an author of a number of references cited in this topic.
Shayna Sarosiek, MD
Assistant Professor
Harvard Medical SchoolBostonUSA
利益声明
SS has received research and consulting funding from BeiGene and ADC Therapeutics.
鉴别诊断
- 多发性骨髓瘤 (Multiple Myeloma, MM)
- 低级别 B 细胞淋巴瘤（例如，滤泡性淋巴瘤）
- 慢性淋巴细胞性白血病 (CLL)
更多 鉴别诊断指南
- Waldenström 巨球蛋白血症诊断和治疗：妙佑巨球蛋白血症分层和风险适应治疗 (Mayo Stratification of Macroglobulinemia and Risk-adapted Therapy, mSMART) 指南
- 肿瘤学临床实践指南：造血细胞移植（HCT）
更多 指南
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