BMJ Best Practice
最后审核时间： 六月 2019
最近更新时间： 三月 2019

小结

定义

病史和查体

关键诊断因素

  • 存在的危险因素
  • 眶周紫癜
  • 巨舌
  • 颈静脉扩张
全部具体信息

其他诊断因素

  • 疲劳
  • 体重减轻
  • 劳力性呼吸困难
  • 疲劳
全部具体信息

危险因素

  • 未定性的单克隆丙种球蛋白病 (MGUS)
  • 炎症性多关节病
  • 慢性感染
  • 炎症性肠病
全部具体信息

诊断性检查

首要检查

  • 血清免疫固定
  • 尿免疫固定
  • 免疫球蛋白游离轻链测定
  • 骨髓活检
全部具体信息

需要考虑的检查

  • 组织活检
  • 淀粉样蛋白沉积物的免疫组织学检查
  • 质谱分析
  • 免疫电子显微镜检查
全部具体信息

治疗流程

疾病持续状态

原发性淀粉样变性 (AL) 和干细胞移植 (SCT) 适应症

原发性淀粉样变性 (AL)，但无法进行 SCT 治疗

非家族性继发性淀粉样变性

家族性继发性淀粉样变性：家族性地中海热

遗传性转甲状腺素蛋白淀粉样变性

贡献者

Morie A. Gertz MD, MACP

Seidler Jr. Professor of Medicine

Consultant in Hematology

Chair Emeritus of the Department of Medicine

Mayo Distinguished Clinician

Mayo Clinic College of Medicine

Rochester

MN

利益冲突披露
MAG declares that he has received honoraria from Celgene Corporation, Prothena Corporation Plc, Onyx Pharmaceuticals, Alnylam, and Ionis Pharmaceuticals. MAG is also an author of several references cited in this monograph.

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Associate Professor of Medicine

Director

Program for Multiple Myeloma and Related Diseases

Princess Margaret Hospital

Toronto

Ontario

Canada

利益冲突披露
DR has been reimbursed by Millennium Pharmaceuticals, Inc and Johnson and Johnson, the manufacturers of bortezomib, for attending several conferences, for speaking at educational meetings, and for consulting work. She has also been reimbursed by Celgene, the manufacturer of lenalidomide and thalidomide, for attending several symposia and serving as a speaker.

Assistant Professor of Medicine and Oncology

Division of Hematology/Oncology

Wayne State University School of Medicine

Barbara Ann Karmanos Cancer Institute

Detroit

MI

利益冲突披露
JZ declares that he has no competing interests.

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