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朗格汉斯细胞组织细胞增生症

最后审阅: 10 Nov 2024
最后更新: 31 Oct 2023

小结

定义

病史和体格检查

关键诊断因素

  • 年龄 <15 岁
  • 骨痛和/或肿胀
  • 皮疹
  • 多尿和烦渴
  • 生长或性成熟障碍
  • 扁平椎
  • 肝脾肿大
完整详情

其他诊断因素

  • 咳嗽和呼吸困难
  • 胸痛
  • 眼眶异常
  • 黄疸
  • 腹水
  • 血细胞减少
  • 发热
  • 厌食
  • 体重减轻
  • 易怒
  • 耳痛
  • 耳分泌物
  • 听力损失
  • 鼓膜穿孔
  • 头痛
  • 神经系统体征
  • 便血
  • 口腔黏膜病变
  • 淋巴结肿大
  • 甲状腺肿大
完整详情

危险因素

  • 吸烟
  • 甲状腺病家族史
  • 围产期感染
完整详情

诊断性检查

首要检查

  • 全血细胞计数 (FBC)
  • 肝功能检查 (LFT)
  • 血清白蛋白
  • 肾功能
  • 血清电解质
  • 血清钙
  • 炎症标志物
  • 内分泌评估(成人)
  • BRAF V600E 突变分析
  • 骨骼检查(儿童)
  • 胸部 X 线检查(儿童)
  • 腹部超声(儿童)
  • 氟脱氧葡萄糖正电子发射断层扫描(FDG-PET)(成人)
  • 造影剂头部 MRI(成人)
  • 组织活检
完整详情

需考虑的检查

  • 内分泌评估(儿童)
  • 凝血功能检查(儿童)
  • 肺功能检查 (PFT)
  • 超声心动图
  • 高分辨率胸部 CT 扫描
  • 听力测定
  • 骨髓穿刺和活检
  • 造影剂头部 MRI(儿童)
  • 脊柱 MRI 检查
  • 头颅 CT
完整详情

治疗流程

急症处理

单系统疾病:累及骨骼

单系统疾病:累及皮肤

单系统疾病:孤立性淋巴结受累

单系统疾病:累及肺脏

单系统疾病:累及 CNS

单系统疾病:神经退行性疾病

多系统疾病

持续性治疗

复发/难治性疾病

撰稿人

作者

Gaurav Goyal, MD

Assistant Professor of Medicine

University of Alabama at Birmingham

Department of Medicine

Division of Hematology/Oncology

Birmingham

AL

利益声明

GG has been reimbursed for advisory board by Opna Bio LLC, and receives royalties from UpToDate.

鸣谢

Dr Gaurav Goyal would like to gratefully acknowledge Dr Oussama Abla and Professor Kimo Stine, previous contributors to this topic.

利益声明

OA declares that he has no competing interests. KS declares that he serves on the Board of the Histiocytosis Association (a non-profit organization), has provided advice for a legal firm reviewing a case of LCH, and receives honoraria for membership of advisory boards to Pfizer and Behring.

同行评议者

R. Maarten Egeler, MD, PhD

Director of Pediatric Immunology, Hematology, Oncology, Bone Marrow Transplantation, and Autoimmune Diseases

Leiden University Medical Center

Leiden

The Netherlands

利益声明

RME has previously worked with Dr Abla on a review of LCH. RME is an author of several references cited in this topic.

Rima Jubran, MD, MPH

Assistant Professor of Pediatrics

Keck School of Medicine

University of Southern California

Children's Hospital Los Angeles

Los Angeles

CA

利益声明

RJ declares that she has no competing interests.

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