MCID: HCL001
MIFTS: 43

Hcl-V malady

Rare diseases, Cancer diseases, Blood diseases categories

Aliases & Classifications for Hcl-V

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Sources:
9Disease Ontology, 47Orphanet, 60UMLS, 41NIH Rare Diseases, 11DISEASES, 43Novoseek, 38NCIt, 55SNOMED-CT, 27ICD9CM, 33MeSH, 26ICD10 via Orphanet, 61UMLS via Orphanet, 34MESH via Orphanet, 25ICD10
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Hcl-V, Aliases & Descriptions:

Name: Hcl-V 9 47
Hairy Cell Leukemia 9 41 11 47 60
Hairy Cell Leukemia Variant 9 47 60
Leukemic Reticuloendotheliosis 41 47
Hcl 41 47
 
Prolymphocytic Variant of Hairy Cell Leukemia 47
Leukemic Reticuloendotheliosis Variant 47
Prolymphocytic Variant of Hcl 47
Hairy Cell Leukaemia Variant 9
Leukemia Hairy Cell 43


Classifications:

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


Characteristics (Orphanet epidemiological data):

47
hairy cell leukemia variant:
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (United States); Age of onset: Adult; Age of death: adult
hairy cell leukemia:
Prevalence: 1-9/1000000 (Europe),1-9/100000 (Europe),1-9/1000000 (United States); Age of onset: Adult


External Ids:

Disease Ontology9 DOID:713, DOID:285
NCIt38 C7401, C7402
ICD9CM27 202.4
MeSH33 D007943
Orphanet47 300878, 58017
ICD10 via Orphanet26 C91.4
UMLS via Orphanet61 C0349633, C0023443
MESH via Orphanet34 D007943
ICD1025 C91.4

Summaries for Hcl-V

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NIH Rare Diseases:41 Hairy cell leukemia is a rare, slow-growing cancer of the blood in which the bone marrow makes too many b cells (lymphocytes), a type of white blood cell that fights infection. the condition is named after these excess b cells which look 'hairy' under a microscope. as the number of leukemia cells increases, fewer healthy white blood cells, red blood cells and platelets are produced. the underlying cause of this condition is unknown. while there is no cure, treatment can lead to remission which can last for years.   last updated: 4/3/2012

MalaCards based summary: Hcl-V, also known as hairy cell leukemia, is related to chronic lymphocytic leukemia and intrapelvic lymph node leukemic reticuloendotheliosis, and has symptoms including splenomegaly, leukemia and leukocytosis. An important gene associated with Hcl-V is BRAF (v-raf murine sarcoma viral oncogene homolog B). The drugs interferon alfa-2a and interferon alfa-2b and the compounds alanine and fatty acid have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, bone and b cells.

Related Diseases for Hcl-V

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

(show top 50)    (show all 156)
idRelated DiseaseScoreTop Affiliating Genes
1chronic lymphocytic leukemia10.7
2intrapelvic lymph node leukemic reticuloendotheliosis10.7
3b-cell lymphomas10.6
4prolymphocytic leukemia10.5
5sarcoma10.5
6vasculitis10.5
7polycythemia vera10.5
8polycythemia10.5
9hodgkin lymphoma10.5
10splenic manifestation of hairy cell leukemia10.5
11hairy cell leukemia of spleen10.5
12splenic marginal zone lymphoma10.5
13gilles de la tourette syndrome10.4
14hyperparathyroidism10.4
15hemolytic anemia10.4
16mantle cell lymphoma10.4
17myelofibrosis10.4
18autoimmune hemolytic anemia10.4
19meningitis10.4
20myeloma10.4
21splenomegaly10.4
22aplastic anemia10.3
23lymphoid leukemia10.3
24marginal zone b-cell lymphoma10.3
25polyclonal hypergammaglobulinemia10.3
26macroglobulinemia10.3
27sporotrichosis10.3
28adenocarcinoma10.3
29dendritic cell sarcoma10.3
30guillain-barre syndrome10.3
31retinitis10.3
32sideroblastic anemia10.3
33acute febrile neutrophilic dermatosis10.3
34essential thrombocythemia10.3
35large granular lymphocyte leukemia10.3
36mycobacterium kansasii10.3
37refractory hairy cell leukemia10.3
38myeloid leukemia10.3
39prostatitis10.3
40attention deficit-hyperactivity disorder10.3
41pol iii-related leukodystrophies10.3
42myocardial infarction10.2
43porphyria cutanea tarda10.2
44melkersson-rosenthal syndrome10.2
45aspergillosis10.2
46lung cancer10.2
47bacteremia10.2
48renal cell carcinoma10.2
49glomerulonephritis10.2
50hematopoietic stem cell transplantation10.2

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



Diseases related to hcl-v

Symptoms for Hcl-V

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Symptoms:

 47 (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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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:

31
Bone marrow, Bone, B cells, Lung, Prostate, Heart, Skin, 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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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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Sources:
43Novoseek
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Compounds related to Hcl-V according to GeneCards Suite gene sharing:

idCompoundScoreTop Affiliating Genes
1alanine439.3NDUFAB1, BRAF
2fatty acid439.2NDUFAB1, BRAF
3aspartate439.0NDUFAB1, BRAF

GO Terms for genes affiliated with Hcl-V

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Molecular functions related to Hcl-V according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1calcium ion bindingGO:00055099.3NDUFAB1, BRAF

Products for genes affiliated with Hcl-V

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies
  • Proteins
  • Kits and Assays

Sources for Hcl-V

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet