小结
定义
病史和体格检查
关键诊断因素
- 存在的危险因素
- 血栓的特征
其他诊断因素
- 出血的特征
- 无症状
- 头痛
- 全身无力/疲乏
- 瘙痒
- 盗汗和骨痛
- 红斑性肢痛症
- 手指、手掌、脚趾、脚跟发红
- 面色发红
- 脾肿大
危险因素
- 受影响的家庭成员
- 年龄＞40 岁
- Budd-Chiari 综合征（Budd-Chiari Syndrome, BCS）
诊断性检查
首要检查
- 血红蛋白
- 红细胞比容
- 白细胞（WBC）计数
- 血小板计数
- 平均红细胞体积 (MCV)
- 肝功能检查 (LFTs)
- JAK2 基因突变筛查
需考虑的检查
- 血氧饱和度
- 血红蛋白-氧亲和力（P50）
- 血清促红细胞生成素
- 红细胞（RBC）总量
- 检测除 JAK2 之外的基因组异常
- 血清铁蛋白
- 血清尿酸
- 腹部超声
- 内源性红系集落
- 骨髓活检
- 血管成像
治疗流程
非妊娠成人：血栓风险较低/中等，无显著的血小板增多
非妊娠成人：血栓风险高，无显著的血小板增多
非妊娠成人：显著血小板增多
妊娠
儿童
非妊娠患者：不耐受其他治疗药物（包括羟基脲、芦可替尼和干扰素）、高龄或者预期寿命短
撰稿人
作者
Prithviraj Bose, MD
Associate Professor
Leukemia Department
Division of Cancer Medicine
The University of Texas MD Anderson Cancer Center
Houston
TX
利益声明
PB has consulted for Incyte and delivered lectures and other educational events for Incyte and Celgene (now BMS). He has participated in advisory boards for Incyte, Celgene (now BMS), Blueprint Medicines, CTI Biopharma and Kartos. PB has received grants from Incyte, Celgene (now BMS), Blueprint Medicines, CTI Biopharma, Kartos, Constellation, Astellas, Pfizer, NS Pharma and Promedior.
Srdan Verstovsek, MD, PhD
Professor
Department of Leukemia
Division of Cancer Medicine
The University of Texas MD Anderson Cancer Center
Houston
TX
利益声明
SV declares that he has no competing interests.
鸣谢
Dr Prithviraj Bose and Dr Srdan Verstovsek would like to gratefully acknowledge Dr Constantine Tam, Dr Scott J. Samuelson, and Dr Josef T. Prchal, previous contributors to this topic.
利益声明
CT, SJS, and JTP declare that they have no competing interests.
同行评议者
Christopher Pechlaner, MD
Associate Professor of Medicine
Innsbruck Medical University
Innsbruck
Austria
利益声明
CP declares that he has no competing interests.
Ross Levine, MD
Assistant Member
Human Oncology and Pathogenesis Program Leukemia Service
Department of Medicine
Memorial Sloan Kettering Cancer Center
New York
NY
利益声明
None declared.
鉴别诊断
- 由低氧引起的继发性红细胞增多
- 原发性血小板增多症 (ET)
- 慢性髓细胞白血病 (CML)
更多 鉴别诊断指南
- NCCN 肿瘤学临床实践指南：骨髓增殖性肿瘤
- 真性红血球增多症的诊断和管理
更多 指南
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