MCID: HCL001
MIFTS: 33

Hcl-V malady

Categories: Blood diseases, Rare diseases, Cancer diseases, Immune diseases

Aliases & Classifications for Hcl-V

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Sources:
11Disease Ontology, 13DISEASES, 52Orphanet, 48Novoseek, 66UMLS, 43NCIt, 29ICD10 via Orphanet, 67UMLS via Orphanet, 60SNOMED-CT
See all MalaCards sources

Aliases & Descriptions for Hcl-V:

Name: Hcl-V 11 13 52
Hairy Cell Leukemia Variant 11 52 66
Prolymphocytic Variant of Hairy Cell Leukemia 52
Leukemic Reticuloendotheliosis Variant 52
 
Prolymphocytic Variant of Hcl 52
Hairy Cell Leukaemia Variant 11
Leukemia Hairy Cell 48

Characteristics:

Orphanet epidemiological data:

52
hairy cell leukemia variant:
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (United States); Age of onset: Adult; Age of death: adult

Classifications:



External Ids:

Disease Ontology11 DOID:713
NCIt43 C7401
Orphanet52 ORPHA300878
SNOMED-CT60 277568007, 54087003
ICD10 via Orphanet29 C91.4
UMLS via Orphanet67 C0349633

Summaries for Hcl-V

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MalaCards based summary: Hcl-V, also known as hairy cell leukemia variant, is related to splenic marginal zone lymphoma and lymphoma, and has symptoms including splenomegaly, leukemia and abdominal pain. An important gene associated with Hcl-V is IGHV4-34 (Immunoglobulin Heavy Variable 4-34), and among its related pathways are Reelin Pathway (Cajal-Retzius cells) and Dendritic Cells Developmental Lineage Pathway. Affiliated tissues include skin, prostate and lung, and related mouse phenotype hematopoietic system.

Related Diseases for Hcl-V

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Graphical network of diseases related to Hcl-V:



Diseases related to hcl-v

Symptoms for Hcl-V

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HPO human phenotypes related to Hcl-V:

(show all 18)
id Description Frequency HPO Source Accession
1 splenomegaly hallmark (90%) HP:0001744
2 leukemia hallmark (90%) HP:0001909
3 abdominal pain hallmark (90%) HP:0002027
4 respiratory insufficiency hallmark (90%) HP:0002093
5 lymphocytosis typical (50%) HP:0100827
6 renal insufficiency occasional (7.5%) HP:0000083
7 skin rash occasional (7.5%) HP:0000988
8 hypermelanotic macule occasional (7.5%) HP:0001034
9 congestive heart failure occasional (7.5%) HP:0001635
10 hemolytic anemia occasional (7.5%) HP:0001878
11 abnormality of coagulation occasional (7.5%) HP:0001928
12 vasculitis occasional (7.5%) HP:0002633
13 lymphadenopathy occasional (7.5%) HP:0002716
14 elevated hepatic transaminases occasional (7.5%) HP:0002910
15 abnormality of macrophages occasional (7.5%) HP:0004311
16 bone marrow hypocellularity occasional (7.5%) HP:0005528
17 neoplasm of the lung occasional (7.5%) HP:0100526
18 prostate neoplasm occasional (7.5%) HP:0100787

Drugs & Therapeutics for Hcl-V

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Drugs for Hcl-V (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
rituximabPhase 21611174722-31-710201696
Synonyms:
AntiCD20
IDEC-102
IDEC-C2B8
 
Ig gamma-1 chain C region
MabThera
Mabthera
Rituxan
rituximab
2interferonsPhase 22112
3
CladribinePhase 2694291-63-820279
Synonyms:
(2R,3S,5R)-5-(6-amino-2-Chloropurin-9-yl)-2-(hydroxymethyl)oxolan-3-ol
(2R,3S,5R)-5-(6-amino-2-chloropurin-9-yl)-2-(hydroxymethyl)oxolan-3-ol
2 Chlorodeoxyadenosine
2'-Deoxy-2-chloroadenosine
2-CdA
2-Chloro-2'-deoxy-beta-adenosine
2-Chloro-2'-deoxyadenosine
2-Chloro-6-amino-9-(2-deoxy-beta-D-erythropentofuranosyl)purine
2-Chlorodeoxyadenosine
2-chloro-6-amino-9-(2-Deoxy-beta-D-erythro-pentofuranosyl)purine
2-chloro-6-amino-9-(2-deoxy-beta-D-erythro-pentofuranosyl)purine
2-chloro-Deoxyadenosine
2-chloro-deoxyadenosine
24757-90-2
2CdA
2ClAdo
4291-63-8
AC-7591
AC1L2FXP
Adenosine, 2-chloro-2'-deoxy
BRN 0624220
C10H12ClN5O3.C3H8
CHEBI:567361
CHEMBL1619
CID20279
CL9
CPD000058553
Chlorodeoxyadenosine
Cladarabine
Cladaribine
Cladribina
Cladribine
Cladribine (JAN/USAN/INN)
Cladribine [USAN:INN:BAN]
Cladribinum
 
CldAdo
D01370
D017338
DB00242
FT-0080707
HMS2052K13
HSDB 7564
LS-15109
Leustat
Leustatin
Leustatin (TN)
Leustatin, 2-chlorodeoxyadenosine, Cladribine
Litak
MLS000028377
MLS000028484
MLS000759397
MLS001077345
MolPort-002-054-532
MolPort-005-935-074
Movectro
Mylinax
NCGC00022567-05
NCGC00164384-01
NSC 105014
NSC 105014-F
NSC-105014
RWJ 26251
RWJ-26251
RWJ-26251-000
S1199_Selleck
SAM001246526
SMR000058553
UNII-47M74X9YT5
ZINC03798064
cladribina
cladribine
cladribinum
4tyrosineNutraceuticalPhase 2633

Interventional clinical trials:

idNameStatusNCT IDPhase
1Treatment of Hairy Cell Leukaemia Variant and Relapsing Hairy Cell Leukaemia With Cladribine Plus RituximabCompletedNCT02157181Phase 2
2Ibrutinib in Treating Patients With Relapsed Hairy Cell LeukemiaRecruitingNCT01841723Phase 2

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Genetic Tests for Hcl-V

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Anatomical Context for Hcl-V

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MalaCards organs/tissues related to Hcl-V:

34
Skin, Prostate, Lung, Heart, Bone, Bone marrow

Animal Models for Hcl-V or affiliated genes

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MGI Mouse Phenotypes related to Hcl-V:

39
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053977.5ANXA1, CD19, CD22, GPR161, ITGAE

Publications for Hcl-V

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Articles related to Hcl-V:

idTitleAuthorsYear
1
Efficient vibrational and translational excitations of a solid metal surface: State-to-state time-of-flight measurements of HCl(v=2,J=1) scattering from Au(111). (19063576)
2008
2
Optical control of ground-state atomic orbital alignment: Cl(2P3/2) atoms from HCl(v=2,J=1) photodissociation. (17935395)
2007
3
Complete remission of hairy cell leukemia variant (HCL-v) complicated by red cell aplasia post treatment with rituximab. (16266917)
2005
4
Proliferative Hairy Cell Leukaemia (HCL-v) Resistant to Alpha-Interferon: Clinical, Diagnostic and In-Vitro Cellular Characteristics. (20394559)
1990

Variations for Hcl-V

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Expression for genes affiliated with Hcl-V

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Search GEO for disease gene expression data for Hcl-V.

Pathways for genes affiliated with Hcl-V

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GO Terms for genes affiliated with Hcl-V

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Sources for Hcl-V

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet