PedAM

Pediatric Disease Annotations & Medicines


	angioimmunoblastic lymphadenopathy with dysproteinemia

Disease ID	1056
Disease	angioimmunoblastic lymphadenopathy with dysproteinemia
Definition	A disorder characterized by proliferation of arborizing small vessels, prominent immunoblastic proliferations and amorphous acidophilic interstitial material. Clinical manifestations include fever, sweats, weight loss, generalized lymphadenopathy and frequently hepatosplenomegaly.
Synonym	[m] angioimmunoblastic lymphadenopathy [m]angioimmunoblastic lymphadenopathy aild ailt angioblastic lymphadenopathy angioimmunoblastic lymphadenopathies angioimmunoblastic lymphadenopathy angioimmunoblastic lymphadenopathy (morphologic abnormality) angioimmunoblastic lymphadenopathy type t-cell lymphoma angioimmunoblastic lymphadenopathy with dysproteinaemia angioimmunoblastic lymphadenopathy with dysproteinemia (aild) angioimmunoblastic lymphadenopathy with dysproteinemia (disorder) angioimmunoblastic lymphoma angioimmunoblastic t-cell lymphoma angioimmunoblastic t-cell lymphoma (disorder) angioimmunoblastic t-cell lymphoma (morphologic abnormality) immunoblastic lymphadenopathies immunoblastic lymphadenopathy immunoblastic lymphadenopathy [disease/finding] lymphadenopathies, angioimmunoblastic lymphadenopathies, immunoblastic lymphadenopathy, angioimmunoblastic lymphadenopathy, angioimmunoblastic, with dysproteinemia lymphadenopathy, immunoblastic peripheral t-cell lymphoma, aild (angioimmunoblastic lymphadenopathy with dysproteinaemia) peripheral t-cell lymphoma, aild (angioimmunoblastic lymphadenopathy with dysproteinemia)
Orphanet	ORPHANET:86886
ICD10	ICD10CM:C86.5
UMLS	C0020981
MeSH	D007119
SNOMED-CT	SNOMEDCT_US_2016_09_01:413537009
Comorbidity	UMLS \| Disease \| Sentences' Count(Total Sentences:26) C0024299 \| lymphoma \| 4 C0079731 \| b-cell lymphoma \| 3 C0079744 \| diffuse large b-cell lymphoma \| 2 C0079731 \| b-cell lymphomas \| 2 C0024299 \| lymphomas \| 2 C0019829 \| hodgkin's lymphoma \| 1 C0035078 \| renal failure \| 1 C0079731 \| b cell lymphoma \| 1 C0242584 \| autoimmune thrombocytopenia \| 1 C0272286 \| immune thrombocytopenia \| 1 C0023418 \| leukemia \| 1 C0031036 \| polyarteritis nodosa \| 1 C0024291 \| hemophagocytic lymphohistiocytosis \| 1 C0002878 \| hemolytic anemia \| 1 C0272126 \| evans syndrome \| 1 C0002871 \| anemia \| 1 C0032285 \| pneumonia \| 1 C0034902 \| pure red cell aplasia \| 1 C0030807 \| pemphigus \| 1 C1522378 \| large granular lymphocytosis \| 1 C0151436 \| leukocytoclastic vasculitis \| 1 C0023484 \| plasma cell leukemia \| 1 C0281963 \| red cell aplasia \| 1 C0026764 \| plasma cell myeloma \| 1 C0085278 \| antiphospholipid syndrome \| 1 C0002880 \| autoimmune hemolytic anemia \| 1
Curated Gene	Entrez_id \| Symbol \| Resource(Total Genes:4) IDH2 \| 3418 \| CTD_human DNMT3A \| 1788 \| CTD_human TET2 \| 54790 \| CTD_human RHOA \| 387 \| CTD_human
Inferring Gene	(Waiting for update.)
Text Mined Gene	(Waiting for update.)
Locus	(Waiting for update.)

Disease ID	1056
Disease	angioimmunoblastic lymphadenopathy with dysproteinemia
Integrated Phenotype	(Waiting for update.)
Text Mined Phenotype	HPO \| Name \| Sentences' Count(Total Phenotypes:16) HP:0012191 \| B-cell lymphoma \| 4 HP:0002665 \| Lymphoma \| 4 HP:0002716 \| Lymph node hyperplasia \| 3 HP:0002664 \| Neoplasia \| 2 HP:0002958 \| Immune dysregulation \| 1 HP:0001880 \| Eosinophilia \| 1 HP:0002090 \| Pneumonia \| 1 HP:0001878 \| Haemolytic anaemia \| 1 HP:0001909 \| Leukemia \| 1 HP:0001903 \| Anemia \| 1 HP:0000083 \| Renal insufficiency \| 1 HP:0012189 \| Hodgkin disease \| 1 HP:0001890 \| Autoimmune hemolytic anemia \| 1 HP:0012410 \| Pure red cell aplasia \| 1 HP:0001973 \| Autoimmune thrombocytopenia \| 1 HP:0030150 \| Plasmacytosis \| 1

Disease ID	1056
Disease	angioimmunoblastic lymphadenopathy with dysproteinemia
Manually Symptom	(Waiting for update.)
Text Mined Symptom	UMLS \| Name \| Sentences' Count(Total Symptoms:2) C0079731 \| b-cell lymphomas \| 2 C0034902 \| pure red cell aplasia \| 1

Manually Genotype(Total Text Mining Genotypes:0)
(Waiting for update.)

All Snps(Total Genotypes:3)
snpId	pubmedId	geneId	geneSymbol	diseaseId	sourceId	sentence	score	Year	geneSymbol_dbSNP	CHROMOSOME	POS	REF	ALT
rs113488022	24689848	387	RHOA	umls:C0020981	BeFree	They include the V600E BRAF mutation in hairy cell leukemia, the L265P MYD88 mutation in Waldenström macroglobulinemia, the G17V RHOA mutation in angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, not otherwise specified, and the Y640F//D661Y/V/H/I//N647I STAT3 mutations in T-cell large granular lymphocytic leukemia.	0.121357209	2014	BRAF	7	140753336	A	T,G,C
rs371896760	24689848	387	RHOA	umls:C0020981	BeFree	They include the V600E BRAF mutation in hairy cell leukemia, the L265P MYD88 mutation in Waldenström macroglobulinemia, the G17V RHOA mutation in angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, not otherwise specified, and the Y640F//D661Y/V/H/I//N647I STAT3 mutations in T-cell large granular lymphocytic leukemia.	0.121357209	2014	ACAA1;MYD88	3	38138714	G	T
rs387907272	24689848	387	RHOA	umls:C0020981	BeFree	They include the V600E BRAF mutation in hairy cell leukemia, the L265P MYD88 mutation in Waldenström macroglobulinemia, the G17V RHOA mutation in angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, not otherwise specified, and the Y640F//D661Y/V/H/I//N647I STAT3 mutations in T-cell large granular lymphocytic leukemia.	0.121357209	2014	MYD88	3	38141150	T	C

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
C0020981	phenytoin	D010672	57-41-0	immunoblastic lymphadenopathy	MESH:D007119	marker/mechanism	7336147

FDA approved drug and dosage information(Total Drugs:0)
(Waiting for update.)

FDA labeling changes(Total Drugs:0)
(Waiting for update.)

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