Langerhans cell histiocytosis is an inflammatory myeloid neoplasm characterised by activating mutations of the mitogen-activated protein kinase pathway. There is clonal proliferation of pathogenic Langerhans cells in single or multiple organs.
有相应临床表现时，病变细胞组织活检显示 CD1a 和Langerhans 蛋白 (CD207) 阳性即可确诊。
Single-system disease may resolve without treatment and require careful monitoring only.
Single-system disease involving risk organs and multi-system disease is treated with systemic chemotherapy.
Adverse prognostic factors include poor response to therapy and risk-organ involvement.
Langerhans cell histiocytosis (LCH) is a rare disease characterised by the clonal proliferation of pathogenic Langerhans cells, leading to inflammation and tissue destruction.
LCH primarily affects children. The most commonly involved organs in children are the bone, skin, pituitary gland, liver, spleen, haematopoietic system, lungs, lymph nodes, and central nervous system.
LCH was previously called histiocytosis X.
- 年龄 <15 岁
- 全血细胞计数 (FBC）
- 肝功能检查 (LFT)
- inflammatory markers
- endocrine evaluation (adults)
- BRAF V600E mutation analysis (adults)
- chest x-ray (children)
- abdominal ultrasound (children)
- fluorodeoxyglucose positron emission tomography (FDG-PET) (adults)
- MRI head with contrast (adults)
- endocrine evaluation (children)
- 肺功能检查 (PFT)
- 高分辨率胸部 CT 扫描
- MRI head with contrast (children)
- 脊柱 MRI 检查
- 头颅 CT
single-system disease: isolated lymph node involvement
single-system disease: neurodegenerative disease
Gaurav Goyal, MD
Assistant Professor of Medicine
University of Alabama at Birmingham
Department of Medicine
Division of Hematology/Oncology
GG is an author of a number of references cited in this topic.
Dr Gaurav Goyal would like to gratefully acknowledge Dr Oussama Abla and Professor Kimo Stine, previous contributors to this topic.
OA declares that he has no competing interests. KS declares that he serves on the Board of the Histiocytosis Association (a nonprofit 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.
R. Maarten Egeler, MD, PhD
Director of Pediatric Immunology, Hematology, Oncology, Bone Marrow Transplantation, and Autoimmune Diseases
Leiden University Medical Center
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
RJ declares that she has no competing interests.
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