DiseaseID 4232
窦组织细胞增生症
disease
H syndrome is a systemic inherited histiocytosis, with characteristic cutaneous findings accompanying systemic manifestations. H syndrome refers to the major clinical findings of hyperpigmentation, hy
Relationship Network
Interactive first-hop connections across herbs, ingredients, formulas, targets, diseases, symptoms, syndromes, evidence, and monographs.
Click a node to open it in a new tab
Disease: 1Symptom: 12Target: 12Links: 24
Arranging relationship network...
Record Fields
Scalar fields from the final disease record.
- Disease Id
- 4232
- Core Entity Id
- 60550
- Source Entity Count
- 1
- Preferred Name
- Sinus Histiocytosis
- Name Cn
- 窦组织细胞增生症
- Name Pinyin
- Dou Zu Zhi Xi Bao Zeng Sheng Zheng
- Name En
- Sinus Histiocytosis
- Name Latin
- Bilingual Status
- complete
- Disease Type
- disease
- Umls Disease Type
- Disease or Syndrome
- Disgenet Type
- disease
- Mesh Class
- Hemic and Lymphatic Diseases
- Do Class
- Hpo Class
- Mesh Class Name
- Hemic and Lymphatic Diseases
- Hpo Class Name
- Do Class Name
- Disease Definition
- H syndrome is a systemic inherited histiocytosis, with characteristic cutaneous findings accompanying systemic manifestations. H syndrome refers to the major clinical findings of hyperpigmentation, hy
- Version
- v1
- Suppressed
- No
Names
Preferred names, aliases, and source labels retained in the final schema.
Name
Sinus Histiocytosis
Role
preferred
Name
H Syndrome
Role
preferred
Name
Sea-Blue Histiocytosis
Role
preferred
Name
Histiocytosis, Sinus
Role
alias
Cross References
Trusted external identifiers retained for this final record.
Herb
HBDIS001384
Me Sh
D015618
Umls
C0019625C0036489
Icd10
D76.3
Sym Map
SMDE00630SMDE00914SMDE13174
Dis Ge Net
C0019625
Orphanet
158029168569
Umls Sty
T047
Me Sh Class
C15
Tcmbank Disease
30184
Attributes
Merged source attributes and domain-specific metadata.
Version
v1v1,v2v2
Suppress
0
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disease Definition
H syndrome is a systemic inherited histiocytosis, with characteristic cutaneous findings accompanying systemic manifestations. H syndrome refers to the major clinical findings of hyperpigmentation, hyNCI2016_02D:A rare disorder of unknown etiology characterized by distention of the lymph node sinuses and sinusoidal histiocytic infiltration. The histiocytes characteristically contain ingested lymphocytes. Patients present with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia. It can affect extranodal sites, including skin, bones, and the respiratory tract. It usually regresses spontaneously.|NCI2016_02D:A morphologic finding indicating the presence of histiocytic infiltrates within distended lymph node sinuses.|MSH2017_2016_08_12:Benign, non-Langerhans-cell, histiocytic proliferative disorder that primarily affects the lymph nodes. It is often referred to as sinus histiocytosis with massive lymphadenopathy.NCI2016_02D:A rare, inherited or acquired syndrome characterized by the presence of histiocytes in the bone marrow which contain granules stained blue with hematoxylin-eosin stain, mild thrombocytopenia and purpura, and splenomegaly.|MSH2017_2016_08_12:A congenital disease caused by an inborn error involving APOLIPOPROTEINS E leading to abnormal LIPID METABOLISM and the accumulation of GLYCOSPHINGOLIPIDS, particularly SPHINGOMYELINS in the HISTIOCYTES. This disorder is characterized by SPLENOMEGALY and the sea-blue histiocytes in the spleen and bone marrow after May Grunwald staining.|HPO2016_07_04:An abnormality of macrophages, also known as histiocytes, in which the cells take on a sea blue appearance due to abnormally increased lipid content. [HPO:probinson, pmid:8797061]
Me Sh Disease Class
Hemic and Lymphatic Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Hemic and Lymphatic Diseases
Umls Semantic Type Name
Disease or Syndrome