HCL-V
MCID: HCL001
MIFTS: 30

Hcl-V (HCL-V) malady

Blood diseases, Cancer diseases, Rare diseases categories
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Summaries for Hcl-V

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MalaCards based summary: Hcl-V, also known as hairy cell leukemia variant, is related to polycythemia vera and polycythemia, and has symptoms including acute abdominal pain/colic, splenomegaly and respiratory distress/dyspnea/respiratory failure/lung volume reduction. An important gene associated with Hcl-V is IGHV4-34 (immunoglobulin heavy variable 4-34). Affiliated tissues include bone marrow, bone and lung.

Aliases & Classifications for Hcl-V

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Sources:
8Disease Ontology, 48Orphanet, 62UMLS, 57SNOMED-CT, 39NCIt, 26ICD10 via Orphanet, 63UMLS via Orphanet
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Hcl-V, Aliases & Descriptions:

Name: Hcl-V 8 48
Hairy Cell Leukemia Variant 8 48 62
Prolymphocytic Variant of Hairy Cell Leukemia 48 62
 
Leukemic Reticuloendotheliosis Variant 48
Prolymphocytic Variant of Hcl 48
Hairy Cell Leukaemia Variant 8


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases, Rare diseases
Anatomical: Blood diseases


Characteristics (Orphanet epidemiological data):

48
hairy cell leukemia variant:
Inheritance: Multigenic/multifactorial; Age of onset: Adulthood; Age of death: Adult


External Ids:

Disease Ontology8 DOID:713
NCIt39 C7401
SNOMED-CT57 277568007, 54087003
ICD10 via Orphanet26 C91.4
UMLS via Orphanet63 C0349633

Related Diseases for Hcl-V

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Diseases related to Hcl-V via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

idRelated DiseaseScoreTop Affiliating Genes
1polycythemia vera10.5
2polycythemia10.5
3chronic myeloid leukemia10.3
4splenic marginal zone lymphoma10.3
5refractory hairy cell leukemia10.3
6myeloid leukemia10.3
7prostatitis10.3
8leukemia10.1
9hairy cell leukemia10.1

Graphical network of diseases related to Hcl-V:



Diseases related to hcl-v

Symptoms for Hcl-V

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Symptoms:

48 (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 20)
id Description Frequency HPO Source Accession
1 splenomegaly hallmark (90%) HP:0001744
2 leukemia hallmark (90%) HP:0001909
3 leukocytosis hallmark (90%) HP:0001974
4 abdominal pain hallmark (90%) HP:0002027
5 respiratory insufficiency hallmark (90%) HP:0002093
6 abnormality of immune system physiology hallmark (90%) HP:0010978
7 lymphocytosis typical (50%) HP:0100827
8 renal insufficiency occasional (7.5%) HP:0000083
9 skin rash occasional (7.5%) HP:0000988
10 hypermelanotic macule occasional (7.5%) HP:0001034
11 congestive heart failure occasional (7.5%) HP:0001635
12 hemolytic anemia occasional (7.5%) HP:0001878
13 abnormality of coagulation occasional (7.5%) HP:0001928
14 vasculitis occasional (7.5%) HP:0002633
15 lymphadenopathy occasional (7.5%) HP:0002716
16 elevated hepatic transaminases occasional (7.5%) HP:0002910
17 abnormality of macrophages occasional (7.5%) HP:0004311
18 bone marrow hypocellularity occasional (7.5%) HP:0005528
19 neoplasm of the lung occasional (7.5%) HP:0100526
20 prostate neoplasm occasional (7.5%) HP:0100787

Drugs & Therapeutics for Hcl-V

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Drug clinical trials:

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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:

32
Bone marrow, Bone, Lung, Prostate, Heart, Monocytes

Animal Models for Hcl-V or affiliated genes

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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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Expression patterns in normal tissues for genes affiliated with Hcl-V

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

Pathways for genes affiliated with Hcl-V

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Compounds for genes affiliated with Hcl-V

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GO Terms for genes affiliated with Hcl-V

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Products for genes affiliated with Hcl-V

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  • Antibodies
  • Proteins
  • Lysates

Sources for Hcl-V

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet