multicentric castleman disease |
Disease ID | 1213 |
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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) |
Inferring Gene | (Waiting for update.) |
Text Mined Gene | (Waiting for update.) |
Locus | (Waiting for update.) |
Disease ID | 1213 |
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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 |
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Disease | multicentric castleman disease |
Manually Symptom | UMLS | Name(Total Manually Symptoms:4) |
Text Mined Symptom | UMLS | Name | Sentences' Count(Total Symptoms:1) |
Manually Genotype(Total Text Mining Genotypes:0) |
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All Snps(Total Genotypes:0) | |
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GWASdb Annotation(Total Genotypes:0) | |
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GWASdb Snp Trait(Total Genotypes:0) | |
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Mapped by lexical matching(Total Items:0) |
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(Waiting for update.) |
Mapped by homologous gene(Total Items:0) |
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(Waiting for update.) |
Chemical(Total Drugs:1) | |||||||||
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CUI | ChemicalName | ChemicalID | CasRN | DiseaseName | DiseaseID | DirectEvidence | PubMedIDs | ||
C1334815 | melphalan | D008558 | 148-82-3 | multi-centric castleman's disease | MESH:C537372 | therapeutic | 17915620 |
FDA approved drug and dosage information(Total Drugs:0) | |
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FDA labeling changes(Total Drugs:0) | |
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