.png){kind=icon}
返回
Histiocytic disorders
.png){kind=icon}
OA
AI
DOI：10.1038/s41572-021-00307-9.png)
.png){kind=icon}
原文求助.png){kind=icon}
分享.png){kind=icon}
收藏
摘要
En 中文
The historic term 'histiocytosis' meaning 'tissue cell' is used as a unifying concept for diseases characterized by pathogenic myeloid cells that share histological features with macrophages or dendritic cells. These cells may arise from the embryonic yolk sac, fetal liver or postnatal bone marrow. Prior classification schemes align disease designation with terminal phenotype: for example, Langerhans cell histiocytosis (LCH) shares CD207(+) antigen with physiological epidermal Langerhans cells. LCH, Erdheim-Chester disease (ECD), juvenile xanthogranuloma (JXG) and Rosai-Dorfman disease (RDD) are all characterized by pathological ERK activation driven by activating somatic mutations in MAPK pathway genes. The title of this Primer (Histiocytic disorders) was chosen to differentiate the above diseases from Langerhans cell sarcoma and malignant histiocytosis, which are hyperproliferative lesions typical of cancer. By comparison LCH, ECD, RDD and JXG share some features of malignant cells including activating MAPK pathway mutations, but are not hyperproliferative. 'Inflammatory myeloproliferative neoplasm' may be a more precise nomenclature. By contrast, haemophagocytic lymphohistiocytosis is associated with macrophage activation and extreme inflammation, and represents a syndrome of immune dysregulation. These diseases affect children and adults in varying proportions depending on which of the entities is involved. Histiocytic disorders are characterized by proliferation of cells with macrophage or dendritic cell phenotype and intense inflammation. Clinical signs and symptoms depend on the specific disease and the tissues and organs affected.
Keyword:
LANGERHANS-CELL HISTIOCYTOSIS
ROSAI-DORFMAN-DISEASE
ERDHEIM-CHESTER DISEASE
FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
MACROPHAGE ACTIVATION SYNDROME
JUVENILE IDIOPATHIC ARTHRITIS
PERIPHERAL LYMPHOID ORGANS
QUALITY-OF-LIFE
MASSIVE LYMPHADENOPATHY
SINUS HISTIOCYTOSIS
期刊
N
IF:
60.6
论文数:
649
被引数:
3.8W
机构
引用论文
Effect of antenna on propagation characteristics of electromagnetic waves in tunnel environments
PREAI
分享收藏Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 MutationsVemurafenib在具有BRAF V600突变的多种非黑色素瘤癌症中的应用
OAAI
OAAI分享收藏BRAFV600E-induced senescence drives Langerhans cell histiocytosis pathophysiology.png)
NATURE MEDICINEOAAI
NATURE MEDICINEIF50
OAAI分享收藏分享收藏
查看更多内容