DiseaseID 4229
莱特勒-西韦病
disease
Hashimoto-Pritzker histiocytosis (HPH) is a variant of Langerhans cell histiocytosis (see this term) characterized by multiple disseminated skin lesions (firm, red-brown, painless papulo-nodules).
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Disease: 1Symptom: 12Target: 12Links: 24
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Record Fields
Scalar fields from the final disease record.
- Disease Id
- 4229
- Core Entity Id
- 60546
- Source Entity Count
- 1
- Preferred Name
- Letterer-Siwe Disease
- Name Cn
- 莱特勒-西韦病
- Name Pinyin
- Lai Te Lei - Xi Wei Bing
- Name En
- Letterer-Siwe Disease
- Name Latin
- Bilingual Status
- complete
- Disease Type
- disease
- Umls Disease Type
- Disease or Syndrome
- Disgenet Type
- disease
- Mesh Class
- Respiratory Tract Diseases; Hemic and Lymphatic Diseases
- Do Class
- disease of anatomical entity
- Hpo Class
- Mesh Class Name
- Respiratory Tract Diseases; Hemic and Lymphatic Diseases
- Hpo Class Name
- Do Class Name
- disease of anatomical entity
- Disease Definition
- Hashimoto-Pritzker histiocytosis (HPH) is a variant of Langerhans cell histiocytosis (see this term) characterized by multiple disseminated skin lesions (firm, red-brown, painless papulo-nodules).
- Version
- v1
- Suppressed
- No
Names
Preferred names, aliases, and source labels retained in the final schema.
Name
Letterer-Siwe Disease
Role
preferred
Name
Granuloma Eosinophilic
Role
preferred
Name
Hashimoto-Pritzker Syndrome
Role
preferred
Name
Histiocytosis, Langerhans-Cell
Role
preferred
Name
Pulmonary Histiocytosis X
Role
preferred
Name
Congenital Langerhans Cell Histiocytosis
Role
alias
Name
Familial Letterer-Siwe Disease
Role
alias
Name
Histiocytosis X, Acute Disseminated
Role
alias
Name
Langerhans-Cell Histiocytosis
Role
alias
Cross References
Trusted external identifiers retained for this final record.
Herb
HBDIS001381HBDIS001693HBDIS014266
Me Sh
D004803D006646
Omim
246400604856
Umls
C0014461C0019621C0023381C1275336C1455705
Icd10
C96.0C96.5C96.6K13.4
Sym Map
SMDE01838SMDE09135SMDE09497SMDE10393SMDE12593
Do Class
DOID:7
Dis Ge Net
C0019621C0023381C1455705
Orphanet
99872
Umls Sty
T047T191
Me Sh Class
C08C15
Tcmbank Disease
192182240924963
Itcmdb Generated
ITX-DISEASE-3F70067A7858ITX-DISEASE-4B5FFEDCCE36ITX-DISEASE-F350BAFD6633
Attributes
Merged source attributes and domain-specific metadata.
Version
v1v2
Suppress
0
Do Class Name
disease of anatomical entity
Disease Type
disease
Do Disease Class
disease of anatomical entity
Umls Disease Type
Disease or SyndromeNeoplastic Process
Disease Definition
Hashimoto-Pritzker histiocytosis (HPH) is a variant of Langerhans cell histiocytosis (see this term) characterized by multiple disseminated skin lesions (firm, red-brown, painless papulo-nodules).NCI2016_02D:A clinical variant of Langerhans cell histiocytosis characterized by unifocal involvement of a bone (most often), skin, or lung. Patients are usually older children or adults usually presenting with a lytic bone lesion. The etiology is unknown. Morphologically, eosinophilic granuloma is characterized by the presence of Langerhans cells in a characteristic milieu which includes histiocytes, eosinophils, neutrophils, and small, mature lymphocytes.|MSH2017_2016_08_12:The most benign and common form of Langerhans-cell histiocytosis which involves localized nodular lesions predominantly of the bones but also of the gastric mucosa, small intestine, lungs, or skin, with infiltration by EOSINOPHILS.|CSP2006:most benign clinical form of Langerhans-cell histiocytosis, which involves localized nodular lesions of the gastric mucosa, small intestine, bones, lungs, or skin, with infiltration by eosinophils; the proliferating cell that appears to be responsible for the clinical manifestations is the Langerhans cell.NCI2016_02D:A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia.NCI2016_NCI-GLOSS_1602D:A group of rare disorders in which too many Langerhans cells (a type of white blood cell) grow in certain tissues and organs including the bones, skin, and lungs, and damage them. Langerhans cell histiocytosis may also affect the pituitary gland (which makes hormones that control other glands and many body functions, especially growth). Langerhans cell histiocytosis is most common in children and young adults.|NCI2016_02D:A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001)|NCI2016_02D:A multifocal, unisystem form of Langerhans-cell histiocytosis. There is involvement of multiple sites in one organ system, most frequently the bone. Patients are usually young children presenting with multiple destructive bone lesions.|MSH2017_2016_08_12:A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.|CSP2006:group of disorders of histiocyte proliferation which includes Letterer-Siwe disease; Hand-Schueller-Christian syndrome; and eosinophilic granuloma; Langerhans cells are components of the lesions.
Me Sh Disease Class
Respiratory Tract Diseases; Hemic and Lymphatic Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Respiratory Tract Diseases; Hemic and Lymphatic Diseases
Umls Semantic Type Name
Disease or SyndromeNeoplastic Process