Langerhans Cell Histiocytosis (LCH)

Alias:

Histiocytosis X

Langerhans Cell Granulomatosis

Histiocytosis, Langerhans-Cell

Langerhans-Cell Histiocytosis

Hashimoto-Pritzger Disease

Letterer-Siwe Disease

Lch

Letterer-Siwe Disease Involving Lymph Nodes of Inguinal Region and Lower Limb

Letterer-Siwe Disease of Lymph Nodes of Inguinal Region Amd/or Lower Limb

Letterer-Siwe Disease of Lymph Nodes of Inguinal Region and/or Lower Limb

Letterer-Siwe Disease of Lymph Nodes of Inguinal Region and Lower Limb

Letterer-Siwe Disease Involving Lymph Nodes of Axilla and Upper Limb

Letterer-Siwe Disease Involving Lymph Nodes of Head, Face, and Neck

Letterer-Siwe Disease Involving Lymph Nodes of Head, Face and Neck

Letterer-Siwe Disease of Lymph Nodes of Axilla and/or Upper Limb

Letterer-Siwe Disease of Lymph Nodes of Head, Face and/or Neck

Letterer-Siwe Disease Involving Lymph Nodes of Multiple Sites

Letterer-Siwe Disease of Lymph Nodes of Axilla and Upper Limb

Letterer-Siwe Disease Involving Intra-Abdominal Lymph Nodes

Letterer-Siwe Disease of Lymph Nodes of Head, Face and Neck

Letterer-Siwe Disease Involving Intrathoracic Lymph Nodes

Letterer-Siwe Disease Involving Intrapelvic Lymph Nodes

Letterer-Siwe Disease of Lymph Nodes of Multiple Sites

Letterer-Siwe Disease of Intra-Abdominal Lymph Nodes

Letterer-Siwe Disease of Intrathoracic Lymph Nodes

Letterer-Siwe Disease of Intrapelvic Lymph Nodes

Letterer-Siwe Disease Involving Spleen

Langerhans-Cell Histiocytosis Nos

Letterer-Siwe Disease of Spleen

Langerhan's Cell Histiocytosis

Familial Letterer-Siwe Disease

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基础信息

疾病表征

基因 & 突变

靶点药物

疾病模型

文献报道

朗格汉斯细胞组织细胞增生症，又称组织细胞增生症X，与非朗格汉斯细胞组织细胞增生症和组织细胞增生症有关，症状包括发热。与朗格汉斯细胞组织细胞增生症相关的重要基因是BRAF（B-Raf原癌基因，丝氨酸/苏氨酸激酶），其相关通路/超级通路包括MIF介导的糖皮质激素调节和MHC I类介导的抗原处理和呈递。在该疾病的背景下，已提到的药物有霉酚酸和抗感染药物。附属组织包括脾脏和骨髓，相关表型为免疫系统和呼吸系统。

Related ID：

MALACARDS：LNG108

OMIM：604856

MESH：C538636

ICD11：216625985