HCL
MCID: HRY003
MIFTS: 62

Hairy Cell Leukemia (HCL) malady

Cancer diseases, Blood diseases categories

Summaries for Hairy Cell Leukemia

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42NIH Rare Diseases, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 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: Hairy Cell Leukemia, also known as leukemic reticuloendotheliosis, is related to leukemia and chronic lymphocytic leukemia. An important gene associated with Hairy Cell Leukemia is BRAF (v-raf murine sarcoma viral oncogene homolog B), and among its related pathways are Downstream signaling in naive CD8+ T cells and Primary immunodeficiency. The drugs interferon alfa-2a and interferon alfa-2b and the compounds pegasys and entecavir have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and bone marrow, and related mouse phenotypes are hematopoietic system and immune system.

Wikipedia:63 Hairy cell leukemia is an uncommon hematological malignancy characterized by an accumulation of abnormal... more...

Aliases & Classifications for Hairy Cell Leukemia

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8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 27ICD9CM, 56SNOMED-CT, 34MeSH, 39NCIt, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 25ICD10
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
hairy cell leukemia:
Prevalence: 1-9/100000; Age of onset: Adulthood


Aliases & Descriptions:

hairy cell leukemia 8 42 10 48 60
leukemic reticuloendotheliosis 42 48
hcl 42 48
leukemia hairy cell 44


External Ids:

Disease Ontology8 DOID:285
ICD9CM27 202.4
MeSH34 D007943
NCIt39 C7402
MESH via Orphanet35 D007943
ICD10 via Orphanet26 C91.4
SNOMED-CT via Orphanet57 118613001, 54087003
UMLS via Orphanet61 C0023443
ICD1025 C91.4

Related Diseases for Hairy Cell Leukemia

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17GeneCards, 18GeneDecks
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Diseases related to Hairy Cell Leukemia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 203)
idRelated DiseaseScoreTop Affiliating Genes
1leukemia32.3ITGAX, PRAME, AICDA, IL2RB, IL2RG, IL3RA
2chronic lymphocytic leukemia31.1ACP5, IL3RA, AICDA, IGHM, ADA
3acute leukemia30.9PRAME, IL3RA, ITGAX
4myeloid leukemia30.8IL3RA
5vasculitis30.7IFNA2, IGHM, ITGAX
6meningitis30.5ADA
7non-hodgkin lymphoma30.5ITGAX, ADA, IL2RB, AICDA
8myelofibrosis30.5IFNA1, IFNA2
9myeloma30.4IGHM, IFNA1, IFNA2, ACP5
10hodgkin's lymphoma30.4IL2RB, AICDA, IGHM, IL3RA
11hepatitis30.4IFNA2, IFNA1, IL2RB
12chronic myeloid leukemia30.4IFNA2, IFNA1, PRAME, ADA
13panuveitis30.2IFNA2
14hepatitis b30.2IFNA1, IGHM, IFNA2
15influenza30.2IFNA1, ADA, IL2RB
16cryoglobulinemia30.2IFNA1, IFNA2
17cutaneous t cell lymphoma30.2ADA, IL2RB, IFNA1, IFNA2
18multiple sclerosis30.2IL2RB, IFNA1, TBX21
19sarcoidosis30.2ADA, IL2RB
20burkitt's lymphoma30.1IGHM, AICDA, IFNA1
21viral hepatitis29.7IFNA1, IFNA2
22hepatitis c29.7IFNA1, AICDA, IGHM, IFNA2
23peritonitis29.7ADA
24refractory hairy cell leukemia10.8
25t-cell leukemia10.7
26hairy cell leukemia of spleen10.6
27prolymphocytic leukemia10.6
28intrapelvic lymph node leukemic reticuloendotheliosis10.5
29acute myeloid leukemia10.5
30hcl-v10.5
31sarcoma10.4
32polycythemia vera10.4
33langerhans cell sarcoma10.4
34natural killer cell leukemia10.4
35autoimmune hemolytic anemia10.4
36mantle cell lymphoma10.4
37splenic marginal zone lymphoma10.4
38hemolytic anemia10.4
39polycythemia10.4
40hyperparathyroidism10.3
41lymphoid leukemia10.3
42macroglobulinemia10.3
43polyclonal hypergammaglobulinemia10.3
44bone lymphoma10.3
45splenic manifestation of hairy cell leukemia10.3
46sporotrichosis10.3
47aplastic anemia10.3
48dendritic cell sarcoma10.3
49guillain-barre syndrome10.3
50marginal zone b-cell lymphoma10.3

Graphical network of the top 20 diseases related to Hairy Cell Leukemia:



Diseases related to hairy cell leukemia

Clinical Features for Hairy Cell Leukemia

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Drugs & Therapeutics for Hairy Cell Leukemia

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Hairy Cell Leukemia

Drug clinical trials:

Search ClinicalTrials for Hairy Cell Leukemia

Search NIH Clinical Center for Hairy Cell Leukemia

Search CenterWatch for Hairy Cell Leukemia

Inferred drug relations via UMLS60/NDF-RT40:

Genetic Tests for Hairy Cell Leukemia

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Anatomical Context for Hairy Cell Leukemia

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32MalaCards
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MalaCards organs/tissues related to Hairy Cell Leukemia:

32
B cells, Bone, Bone marrow, T cells, Spleen, Myeloid, Skin, Monocytes, Liver, Lymph node, Colon, Kidney, Prostate, Cortex, Brain, Pancreas

Animal Models for Hairy Cell Leukemia or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Hairy Cell Leukemia:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000539710.1ITGAX, TBX21, IGHM, AICDA, IL2RB, IL2RG
2MP:00053879.8ADA, BRAF, ITGAX, TBX21, IGHM, AICDA

Publications for Hairy Cell Leukemia

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Sources:
50PubMed
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Articles related to Hairy Cell Leukemia:

(show top 50)    (show all 619)
idTitleAuthorsYear
1
Efficacy of vemurafenib in hairy-cell leukemia. (24428489)
2014
2
Melioidosis and hairy cell leukemia in 2 travelers returning from Thailand. (23750355)
2013
3
Continued response off treatment after BRAF inhibition in refractory hairy cell leukemia. (23690412)
2013
4
Hairy cell leukemia: epidemiology, pharmacokinetics of cladribine, and long-term follow-up of subcutaneous therapy. (21599605)
2011
5
Definition of remission, minimal residual disease, and relapse in hairy cell leukemia bone marrow biopsy histology and immunohistology specimens. (21463123)
2011
6
BRAF mutations in hairy-cell leukemia. (21899462)
2011
7
Modern strategies for hairy cell leukemia. (21220590)
2011
8
Development of cladribine at Scripps for hairy cell leukemia and current results. (21504293)
2011
9
Interferon-alpha is a very effective salvage therapy for patients with hairy cell leukemia relapsing after cladribine: a report of three cases. (20509009)
2011
10
Hairy cell leukemia presenting as a discrete liver mass: diagnosis by fine needle aspiration biopsy. (18181187)
2008
11
Erythema nodosum in association with newly diagnosed hairy cell leukemia and group C streptococcus infection. (18360121)
2008
12
Severe reactivation of hepatitis B virus infection in a patient with hairy cell leukemia: Should lamivudine prophylaxis be recommended to HBsAg-negative, anti-HBc-positive patients? (17033754)
2006
13
2-CdA in the treatment of hairy cell leukemia: a review of long-term follow-up. (17107901)
2006
14
Immunophenotyping and differential diagnosis of hairy cell leukemia. (16990106)
2006
15
Cryptococcus neoformans meningitis in a patient with hairy cell leukemia. (16628737)
2006
16
Immunoglobulin superfamily receptor translocation associated 2 protein on lymphoma cell lines and hairy cell leukemia cells detected by novel monoclonal antibodies. (15671532)
2005
17
Successful treatment of extranodal Hodgkin's lymphoma in a patient with longstanding hairy cell leukemia. (16019516)
2005
18
The value of tartrate resistant acid phosphatase (TRAP) immunoreactivity in diagnosis of hairy cell leukemia. (15799758)
2005
19
Cladribine: from the bench to the bedside--focus on hairy cell leukemia. (15485311)
2004
20
Disruption of a novel ectodermal neural cortex 1 antisense gene, ENC- 1AS and identification of ENC-1 overexpression in hairy cell leukemia. (15459180)
2004
21
The role of autocrine FGF-2 in the distinctive bone marrow fibrosis of hairy-cell leukemia (HCL). (12689941)
2003
22
Delayed suprachoroidal hemorrhage after needle revision of trabeculectomy bleb in a patient with hairy cell leukemia. (14644230)
2003
23
Synaptojanin 2 is recognized by HLA class II-restricted hairy cell leukemia-specific T cells. (14562116)
2003
24
Effective treatment of autoimmune hemolytic anemia and hairy cell leukemia with interferon-alpha. (11982619)
2002
25
The degree of bone marrow infiltration in patients with hairy cell leukemia treated with splenectomy compatible with long-term hematological remission. (11327542)
2001
26
Efficacy of the anti-CD22 recombinant immunotoxin BL22 in chemotherapy-resistant hairy-cell leukemia. (11474661)
2001
27
Clonal chromosome rearrangements in hairy cell leukemia: personal experience and review of literature. (11566344)
2001
28
Secondary acute myeloid leukemia following successful treatment of hairy cell leukemia with cladribine. (10979762)
2000
29
CD5+ immunophenotype in the bone marrow but not in the peripheral blood in a patient with hairy cell leukemia. (11014896)
2000
30
Prolonged interferon-alpha-2b treatment of hairy cell leukemia patients. (10863983)
2000
31
Somatic mutation analysis of IgH variable regions reveals that tumor cells of most parafollicular (monocytoid) B-cell lymphoma, splenic marginal zone B-cell lymphoma, and some hairy cell leukemia are composed of memory B lymphocytes. (10088550)
1999
32
Filgrastim for cladribine-induced neutropenic fever in patients with hairy cell leukemia. (10194424)
1999
33
Cytogenetic findings in five patients with hairy cell leukemia. (10198621)
1999
34
Pathologic fracture occurring 22 years after diagnosis of hairy cell leukemia: case report and literature review. (10214106)
1999
35
Second malignancies in patients with hairy cell leukemia in british columbia: a 20-year experience. (9694703)
1998
36
Long-term follow-up of patients with hairy cell leukemia after cladribine treatment. (9731048)
1998
37
Hairy cell leukemia-variant treated with 2-chlorodeoxyadenosine--a report of three cases. (9168448)
1997
38
Remission of hairy cell leukemia without treatment. (9408771)
1997
39
Hairy-cell leukemia and alpha-interferon treatment: long-term responders. (9175317)
1997
40
Long-term results in hairy cell leukemia treated with 2-chlorodeoxyadenosine. (9389210)
1997
41
Establishment of a new cell line from a patient with hairy cell leukemia-Japanese variant. (9168447)
1997
42
Early age onset of hairy cell leukemia presenting with leukocytosis. (8923794)
1996
43
Persistent clonal excess and skewed T-cell repertoire in T cells from patients with hairy cell leukemia. (8611705)
1996
44
Immunophenotypic features and configuration of immunoglobulin genes in hairy cell leukemia-Japanese variant. (8709650)
1996
45
Leukemic cells in hairy cell leukemia and B cell chronic lymphocytic leukemia release soluble TNF receptors. (7596168)
1995
46
Neutropenic fever following cladribine therapy for symptomatic hairy-cell leukemia: predictive factors and effects of granulocyte-macrophage colony-stimulating factor. (7619752)
1995
47
Hairy cell leukemia: splenectomy after alpha 2b-interferon therapy. (1878598)
1991
48
Biochemical and immunological responses of hairy cell leukemia patients to interferon beta. (1760815)
1991
49
Leukemic macrocheilitis associated with hairy-cell leukemia and the Melkersson-Rosenthal syndrome. (3950137)
1986
50
Hairy cell leukemia with chylous ascites. (578227)
1977

Genetic Variations for Hairy Cell Leukemia

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Expression for genes affiliated with Hairy Cell Leukemia

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Hairy Cell Leukemia

Search GEO for disease gene expression data for Hairy Cell Leukemia.

Pathways for genes affiliated with Hairy Cell Leukemia

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Sources:
37NCBI BioSystems Database, 29KEGG, 12EMD Millipore, 53Reactome, 51QIAGEN
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Pathways related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.4BRAF, IL2RB, IL2RG
210.4ADA, IL2RG, AICDA
3
Hide members
10.4TBX21, IL2RB, IL2RG
4
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10.3IL3RA, IL2RG, IL2RB
5
Immune response IL-2 activation and signaling pathway
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10.3IL3RA, IL2RG, IL2RB
6
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10.3IL3RA, IL2RG, IFNA1
7
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10.3IFNA2, IFNA1, IL2RB, IL2RG
8
Hide members
10.3ITGAX, IL2RB, IL2RG, IL3RA
9
Hide members
10.3BRAF, IFNA2, IFNA1, IGHM, IL2RG
1010.3IL3RA, IL2RG, IL2RB, IFNA1, IFNA2
11
Hide members
10.3IFNA2, IFNA1, IL2RB, IL2RG, IL3RA
1210.3IFNA2, IFNA1, IL2RB, IL2RG, IL3RA
13
Hide members
10.2IL3RA, IL2RG, IL2RB, IGHM, IFNA1, IFNA2

Compounds for genes affiliated with Hairy Cell Leukemia

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Sources:
44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 49PharmGKB, 2BitterDB, 24HMDB
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Compounds related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

(show all 37)
idCompoundScoreTop Affiliating Genes
1pegasys4410.8IFNA1, IFNA2
2entecavir4410.8IFNA1, IFNA2
3pentostatin44 59 28 1113.8IFNA2, IFNA1, ADA
4denileukin diftitox44 49 1112.8IL2RG, IL2RB, IFNA1
5mizoribine4410.8IFNA2, IFNA1
6didanosine44 1111.8IFNA2, IFNA1, ADA
7busulfan44 1111.8ADA, IFNA1, IFNA2
8famciclovir44 1111.8IFNA2, IFNA1
9intron a4410.8IFNA2, IFNA1
10levamisole44 1111.8IL2RB, IFNA1, IFNA2
11n-phosphonacetyl-l-aspartate4410.7IFNA2, IFNA1
122,5-oligoadenylate4410.7ADA, IL2RB, IFNA1, IFNA2
13neopterin4410.7ADA, IL2RB, IFNA1, IFNA2
14Aldesleukin1110.7IL2RG, IL2RB
15polyethylene glycol4410.7IFNA2, IFNA1, ACP5, ADA
16vidarabine44 1111.7IFNA2, ADA
17anagrelide44 1111.7IFNA2, IFNA1
18ribavirin44 49 1112.7ADA, IFNA1, IFNA2
19ifn-alpha4410.7IFNA2, IFNA1, IL2RB
20lomustine44 1111.7IFNA2, IFNA1
215-deoxy-5-fluorouridine4410.7IFNA1, IFNA2
22cyclosporin a44 28 5912.7IFNA1, IL2RB, IL2RG, ACP5, ADA
23aspartate4410.7BRAF, IFNA2, IFNA1, IL2RB, ACP5, ADA
24adefovir4410.6IFNA2, IFNA1
2512-o-tetradecanoylphorbol 13-acetate4410.6BRAF, IFNA2, IL2RB, IL3RA
26cycloheximide4410.6IFNA1, ITGAX, IL2RB, RGS1
27zidovudine44 1111.6ADA, IFNA1, IFNA2
28azathioprine44 49 2 1113.6ACP5, IL2RB, IFNA2
29leukotriene b444 59 2412.5ACP5, IL2RB, ITGAX
30amantadine44 1111.5IFNA1, IFNA2
31retinoic acid44 2411.5BRAF, IFNA2, IFNA1, ITGAX, IL2RB, ADA
32rapamycin4410.5BRAF, IFNA1, IL2RB, ACP5
33cytarabine44 49 1112.4IFNA2, IFNA1, ADA
34glycyrrhizin44 2811.4IL2RB, IFNA1
35nucleoside4410.4IFNA2, IFNA1, ADA
36vincristine44 49 1112.3IFNA2, IFNA1, ADA
37prednisolone44 28 1112.1IL2RB, IFNA1, IFNA2

GO Terms for genes affiliated with Hairy Cell Leukemia

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16Gene Ontology
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Cellular components related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1plasma membraneGO:00588610.0ITGAX, PRAME, IGHM, IL2RB, IL2RG, IL3RA
2external side of plasma membraneGO:00989710.0ADA, IL2RG, IL2RB, ITGAX

Biological processes related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1organ morphogenesisGO:00988710.4SIX6, ITGAX, BRAF
2interleukin-2-mediated signaling pathwayGO:03811010.1IL2RG, IL2RB

Molecular functions related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1interleukin-2 bindingGO:01997610.4IL2RG, IL2RB
2interleukin-2 receptor activityGO:00491110.1IL2RG, IL2RB

Products for genes affiliated with Hairy Cell Leukemia

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  • Antibodies
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Sources for Hairy Cell Leukemia

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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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet