MCID: HCL001
MIFTS: 43

Hcl-V malady

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

Aliases & Classifications for Hcl-V

About this section
Sources:
65UMLS, 10Disease Ontology, 12DISEASES, 51Orphanet, 47Novoseek, 42NCIt, 59SNOMED-CT, 28ICD10 via Orphanet, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Hcl-V:

Name: Hcl-V 10 12 51
Hairy Cell Leukemia Variant 10 51 65
Prolymphocytic Variant of Hairy Cell Leukemia 51
Leukemic Reticuloendotheliosis Variant 51
 
Prolymphocytic Variant of Hcl 51
Hairy Cell Leukaemia Variant 10
Hairy Cell Leukemia 65
Leukemia Hairy Cell 47

Characteristics:

Orphanet epidemiological data:

51
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 Ontology10 DOID:713
NCIt42 C7401
Orphanet51 300878
SNOMED-CT59 277568007, 54087003
ICD10 via Orphanet28 C91.4
UMLS via Orphanet66 C0349633
UMLS65 C0349633

Summaries for Hcl-V

About this section
MalaCards based summary: Hcl-V, also known as hairy cell leukemia variant, is related to methotrexate-associated lymphoproliferation and central pontine myelinolysis, and has symptoms including splenomegaly, leukemia and abdominal pain. An important gene associated with Hcl-V is CCND1 (Cyclin D1), and among its related pathways are C-MYB transcription factor network and Transcription Ligand-dependent activation of the ESR1/SP pathway. The drugs interferon alfa-2a and interferon alfa-2b have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and bone marrow.

Related Diseases for Hcl-V

About this section

Graphical network of the top 20 diseases related to Hcl-V:



Diseases related to hcl-v

Symptoms for Hcl-V

About this section

Symptoms:

 51 (show all 25)
  • acute abdominal pain/colic
  • splenomegaly
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • bone marrow/medullar infiltration
  • hyperleukocytosis/leukocytosis
  • myeloproliferative syndrome/chronic leukemia
  • asthenia/fatigue/weakness
  • anaemia
  • hyperlymphocytosis
  • cutaneous rash
  • macules
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • abnormal hepatic enzymes/transaminases
  • heart/cardiac failure
  • vascularitis/vasculitides/arteritis
  • lymphadenopathy/polyadenopathies
  • renal failure
  • bone marrow failure/pancytopenia
  • hemolytic anemia
  • monocytes/macrophages anomalies
  • hemorrhage/hemorrhagic syndrome/excessive/long-lasting bleeding
  • neoplasms/tumors
  • lung/bronchopulmonary neoplasm/tumor/carcinoma/cancer
  • prostate/prostatic neoplasm/tumor/carcinoma/cancer

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

About this section

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

(show all 8)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Cladribineapproved, investigationalPhase 2674291-63-820279
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
2
rituximabapprovedPhase 21582174722-31-710201696
Synonyms:
AntiCD20
IDEC-102
IDEC-C2B8
 
Ig gamma-1 chain C region
MabThera
Mabthera
Rituxan
rituximab
3interferonsPhase 21930
4Immunosuppressive AgentsPhase 210422
5Immunologic FactorsPhase 218483
62-chloro-3'-deoxyadenosinePhase 229
7Antirheumatic AgentsPhase 28496
8tyrosineNutraceuticalPhase 2596

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

Search NIH Clinical Center for Hcl-V

Inferred drug relations via UMLS65/NDF-RT43:

Genetic Tests for Hcl-V

About this section

Anatomical Context for Hcl-V

About this section

MalaCards organs/tissues related to Hcl-V:

33
Bone, Lung, Bone marrow, Prostate, Heart, Skin, Monocytes

Animal Models for Hcl-V or affiliated genes

About this section

MGI Mouse Phenotypes related to Hcl-V:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes

Publications for Hcl-V

About this section

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

About this section

Expression for genes affiliated with Hcl-V

About this section
Search GEO for disease gene expression data for Hcl-V.

Pathways for genes affiliated with Hcl-V

About this section

GO Terms for genes affiliated with Hcl-V

About this section

Cellular components related to Hcl-V according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:00098979.5ADA, CD5

Biological processes related to Hcl-V according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1response to vitamin EGO:003319710.0ADA, CCND1
2response to organic cyclic compoundGO:00140709.8ACP5, CCND1
3nucleobase-containing small molecule metabolic processGO:00550869.3ADA, DCK
4negative regulation of inflammatory responseGO:00507289.0ACP5, ADA, IL2RA

Sources for Hcl-V

About this section
2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet