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Pediatric Disease Annotations & Medicines



   multicentric castleman disease
  

Disease ID 1213
Disease multicentric castleman disease
Definition
A form of angiofollicular lymphoid hyperplasia characterized by fever, generalized lymphadenopathy, hypergammaglobulinemia, and dysfunction of multiple organs. Other signs and symptoms include anemia, thrombocytopenia, hepatomegaly, peripheral neuropathy and pleural effusions. Morphologically, in the majority of cases the lymph nodes show features of angiofollicular lymphoid hyperplasia of the plasma cell type. In a minority of cases, changes of angiofollicular lymphoid hyperplasia of the hyaline-vascular type are seen. In contrast to patients with localized disease who are usually cured following resection of the lesion, patients with the multicentric form of the disease may follow a progressive clinical course, complicated by infection, Kaposi sarcoma, or lymphoma.
Synonym
castleman's disease, multicentric
hhv-8-associated multicentric castleman disease
hhv8 multicentric castleman disease
idiopathic multicentric castleman's disease
kshv muticentric castleman disease
kshv-associated multicentric castleman disease
multi-centric castleman's disease
multicentric angiofollicular lymphoid hyperplasia
multicentric castleman's disease
multicentric plasma cell variant of castleman's disease
plasmablastic multicentric castleman disease
Orphanet
UMLS
C1334815
Comorbidity
UMLS | Disease | Sentences' Count(Total Sentences:9)
C1261473  |  sarcoma  |  2
C0032285  |  pneumonia  |  1
C0002871  |  anemia  |  1
C0009447  |  common variable immunodeficiency  |  1
C0041408  |  turner syndrome  |  1
C0024299  |  lymphoma  |  1
C0036220  |  kaposi sarcoma  |  1
C0002880  |  autoimmune hemolytic anemia  |  1
C0002878  |  hemolytic anemia  |  1
Curated Gene
Entrez_id | Symbol | Resource(Total Genes:1)
IL6R  |  3570  |  ORPHANET
Inferring Gene(Waiting for update.)
Text Mined Gene(Waiting for update.)
Locus(Waiting for update.)
Disease ID 1213
Disease multicentric castleman disease
Integrated Phenotype(Waiting for update.)
Text Mined Phenotype
HPO | Name | Sentences' Count(Total Phenotypes:10)
HP:0100242  |  Sarcoma  |  2
HP:0001903  |  Anemia  |  1
HP:0002716  |  Lymph node hyperplasia  |  1
HP:0002721  |  Immunodeficiency  |  1
HP:0008940  |  Generalized lymphadenopathy  |  1
HP:0007461  |  Hemangiomatosis  |  1
HP:0001890  |  Autoimmune hemolytic anemia  |  1
HP:0002090  |  Pneumonia  |  1
HP:0002665  |  Lymphoma  |  1
HP:0001878  |  Haemolytic anaemia  |  1
Disease ID 1213
Disease multicentric castleman disease
Manually Symptom
UMLS  | Name(Total Manually Symptoms:4)
C0878544  |  cardiomyopathy
C0206061  |  interstitial pneumonia
C0032131  |  plasmacytoma
C0017609  |  neovascular glaucoma
Text Mined Symptom
UMLS | Name | Sentences' Count(Total Symptoms:1)
Manually Genotype(Total Text Mining Genotypes:0)
(Waiting for update.)
All Snps(Total Genotypes:0)
(Waiting for update.)
GWASdb Annotation(Total Genotypes:0)
(Waiting for update.)
GWASdb Snp Trait(Total Genotypes:0)
(Waiting for update.)
Mapped by lexical matching(Total Items:0)
(Waiting for update.)
Mapped by homologous gene(Total Items:0)
(Waiting for update.)
Chemical(Total Drugs:1)
CUI ChemicalName ChemicalID CasRN DiseaseName DiseaseID DirectEvidence PubMedIDs
C1334815melphalanD008558148-82-3multi-centric castleman's diseaseMESH:C537372therapeutic17915620
FDA approved drug and dosage information(Total Drugs:0)
(Waiting for update.)
FDA labeling changes(Total Drugs:0)
(Waiting for update.)