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骨髓增生异常综合征

最后审阅: 26 Jun 2024
最后更新: 11 Sep 2023

小结

定义

病史和体格检查

关键诊断因素

  • 老年
  • 乏力
  • 运动不耐受
  • 苍白
  • 瘀斑或出血
  • 既往化疗和/或放疗
  • 先天性疾病
  • 细菌感染
完整详情

其他诊断因素

  • 存在的危险因素
  • 自身免疫性疾病
  • 脾肿大
  • 肝肿大
  • 淋巴结肿大
完整详情

危险因素

  • 年龄>70 岁
  • 既往化疗
  • 既往接受过放射治疗
  • 既往自体造血干细胞移植
  • 先天性疾病
  • 烟草
  • 再生障碍性贫血
  • 阵发性睡眠性血红蛋白尿 (PNH)
完整详情

诊断性检查

首要检查

  • FBC 和分类计数
  • 外周血涂片
  • 网织红细胞计数
  • 红细胞内叶酸
  • 血清维生素 B12
  • 铁检测
  • 骨髓穿刺涂片和铁染色
  • 骨髓穿刺活检
  • 基因检测
完整详情

需考虑的检查

  • 病毒血清学
  • 血清促红细胞生成素
  • 乳酸脱氢酶
  • HLA 分型
  • 流式细胞术
完整详情

治疗流程

急症处理

较低风险 MDS:无症状血细胞减少

较低风险 MDS:无 del(5q) 的有症状血细胞减少

较低风险 MDS:有症状血细胞减少伴 del(5q)

较高风险 MDS:移植候选者

较高风险 MDS:非移植候选者

撰稿人

作者

Vijaya Raj Bhatt, MBBS, MS

​Associate Professor

Section Leader, Malignant Hematology

University of Nebraska Medical Center Division of Hematology-Oncology

Nebraska

NE

利益声明

VRB has participated in a Safety Monitoring Committee for Protagonist Therapeutics. He has received consulting fees from Imugene; research funding (institutional) from Abbvie, Pfizer, Incyte, Jazz, and NMDP; and drug support (institutional) from Chimerix for a trial.

Prajwal Dhakal, MBBS

​Clinical Assistant Professor of Internal Medicine-Hematology, Oncology, and Blood and Marrow Transplantation

University of Iowa

Iowa City

IA

利益声明

PD declares that he has no competing interests.

鸣谢

Dr Vijaya Raj Bhatt and Dr Prajwal Dhakal would like to gratefully acknowledge Professor Apar Kishor Ganti and Associate Professor Alissa Marr, previous contributors to this topic.

利益声明

AKG has received research support from Amgen, Apexigen, Bristol-Myers Squibb, Janssen, Merck, New Link Genetics, Pfizer, and Takeda Oncology. AKG has been reimbursed for consulting work for AbbVie and Genentech. None of the grants or payments relate to work involving myelodysplastic syndrome. AM declares that she has no competing interests.

同行评议者

David P. Steensma, MD, FACP

Associate Professor of Medicine (Hematology) and Oncology

Division of Hematology

Department of Medicine

Mayo Clinic

Rochester

MN

利益声明

DPS declares that he has no competing interests.

Adrian C. Newland, BA, MB, BCh, MA, FRCP, FRCPath

Professor of Haematology

Queen Mary University

London

UK

利益声明

ACN declares that he has no competing interests.

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