HCL
MCID: HRY003
MIFTS: 53

Hairy Cell Leukemia (HCL) malady

Cancer, Blood categories

Summaries for Hairy Cell Leukemia

Sources:
43NIH Rare Diseases, 64Wikipedia, 33MalaCards
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NIH Rare Diseases:43 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 acute leukemia and vasculitis. 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. Related mouse phenotypes are hematopoietic system and immune system.

Wikipedia:64 Hairy cell leukemia is an uncommon hematological malignancy characterized by an accumulation of abnormal... more...

Aliases & Classifications for Hairy Cell Leukemia

Sources:
8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 49Orphanet, 61UMLS, 45Novoseek, 27ICD9CM, 57SNOMED-CT, 35MeSH, 40NCIt, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
hairy cell leukemia:
Prevalence: 1-9/100000; Age of onset: Adulthood


Aliases & Descriptions:

hairy cell leukemia 8 43 10 49 61
leukemic reticuloendotheliosis 43 49
hcl 43 49
leukemia hairy cell 45


External Ids:

Disease Ontology8 DOID:285
ICD9CM27 202.4
MeSH35 D007943
NCIt40 C7402
MESH via Orphanet36 D007943
ICD10 via Orphanet26 C91.4
SNOMED-CT via Orphanet58 118613001, 54087003
UMLS via Orphanet62 C0023443
ICD1025 C91.4

Related Diseases for Hairy Cell Leukemia

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 181)
idRelated DiseaseScoreTop Affiliating Genes
1acute leukemia30.9IL3RA, PRAME, ITGAX
2vasculitis30.7IGHM, ITGAX, IFNA2
3splenomegaly30.6ITGAX, IFNA1, IFNA2
4chronic myeloid leukemia30.4IFNA2, IFNA1, PRAME, ADA
5cryoglobulinemia30.2IFNA1, IFNA2
6hepatitis c30.2IFNA2, IFNA1, IGHM, AICDA
7kaposi's sarcoma30.2IFNA1
8b-cell chronic lymphocytic leukemia30.2IL2RB, AICDA, IGHM
9burkitt's lymphoma30.2AICDA, IGHM, IFNA1
10viral hepatitis29.8IFNA1, IFNA2
11t-cell leukemia11.4
12refractory hairy cell leukemia10.8
13leukemia/lymphoma, t-cell10.7
14leukemia, t-cell, chronic10.6
15intrapelvic lymph node leukemic reticuloendotheliosis10.6
16acute myeloid leukemia10.5
17hcl-v10.5
18polycythemia vera10.4
19langerhans cell sarcoma10.4
20acute t cell leukemia10.4
21natural killer cell leukemia10.4
22autoimmune hemolytic anemia10.4
23mantle cell lymphoma10.4
24n syndrome10.4
25splenic marginal zone lymphoma10.4
26lymphoid leukemia10.3
27macroglobulinemia10.3
28polyclonal hypergammaglobulinemia10.3
29bone lymphoma10.3
30splenic manifestation of hairy cell leukemia10.3
31sporotrichosis10.3
32hairy cell leukemia of spleen10.3
33aplastic anemia10.3
34acute febrile neutrophilic dermatosis10.3
35essential thrombocythemia10.3
36waldenstrom macroglobulinemia10.3
37mycobacterium kansasii10.3
38guillain-barr� syndrome10.3
39squamous cell papilloma10.1
40membranoproliferative glomerulonephritis10.1
41antiphospholipid syndrome10.1
42congenital dyserythropoietic anemia10.1
43bone carcinoma10.1
44melkersson-rosenthal syndrome10.1
45neuroendocrine carcinoma10.1
46porphyria cutanea tarda10.1
47lambert-eaton myasthenic syndrome10.1
48cerebritis10.1
49adult t-cell leukemia10.1
50pancytopenia10.1

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



Diseases related to hairy cell leukemia

Clinical Features for Hairy Cell Leukemia

Drugs & Therapeutics for Hairy Cell Leukemia

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

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 UMLS61/NDF-RT41:

Genetic Tests for Hairy Cell Leukemia

Anatomical Context for Hairy Cell Leukemia

Animal Models for Hairy Cell Leukemia or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Hairy Cell Leukemia:

37
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

Sources:
51PubMed
See all sources

Articles related to Hairy Cell Leukemia:

(show top 50)    (show all 619)
idTitleAuthorsYear
1
Clinical characteristics and long-term outcome of young hairy cell leukemia patients treated with cladribine: a single-institution series. (24192579)
2014
2
Successful vemurafenib salvage treatment in a patient with primary refractory hairy cell leukemia and pulmonary aspergillosis. (24337488)
2013
3
Hairy cell leukemia--a rare type of leukemia. A retrospective study on 39 patients. (24068406)
2013
4
Phase I trial of anti-CD22 recombinant immunotoxin moxetumomab pasudotox (CAT-8015 or HA22) in patients with hairy cell leukemia. (22355053)
2012
5
Secondary T-lymphoblastic leukemia in a patient with hairy cell leukemia following cladribine therapy: report of an extremely rare case and review of the literature. (21980957)
2012
6
Variables in the quantification of CD4 in normals and hairy cell leukemia patients. (20687201)
2011
7
Synchronous Detection of Hairy Cell Leukemia and HIV-Negative Kaposi's Sarcoma of the Lymph Node: A Diagnostic Challenge and a Rare Coincidence. (22087095)
2011
8
Mechanism of action of pentostatin and cladribine in hairy cell leukemia. (21463108)
2011
9
Unusual clinical manifestations, rare sites of involvement, and the association of other disorders with hairy cell leukemia. (21599607)
2011
10
Hairy cell leukemia and the microenvironment. (21504289)
2011
11
Very long-term eradication of minimal residual disease in patients with hairy cell leukemia after a single course of cladribine. (20056789)
2010
12
Hairy cell leukemia: current therapies and future directions. (21091144)
2010
13
Importance of minimal residual disease in hairy cell leukemia: monoclonal antibodies as a therapeutic strategy. (19814695)
2009
14
Historical aspects and milestones in the development of effective treatment for hairy cell leukemia. (19814690)
2009
15
Hairy-cell leukemia with inaugural joint manifestations. (19541526)
2009
16
Hairy cell leukemia presenting as multiple discrete hepatic lesions. (19764101)
2009
17
T-box-expressed-in-T-cells (T-bet) expression by the tumor cells of hairy-cell leukemia correlates with interferon-gamma production. (19757302)
2009
18
Teenager with hairy cell leukemia: 30-year follow-up. (19047276)
2009
19
Selective influences in the expressed immunoglobulin heavy and light chain gene repertoire in hairy cell leukemia. (18387977)
2008
20
Hairy cell leukemia. (18283787)
2008
21
A novel CD11c monoclonal antibody effective in formalin-fixed tissue for the diagnosis of hairy cell leukemia. (18580071)
2008
22
Role of interferon-alpha administration after 2-deoxycoformycin in the treatment of hairy cell leukemia patients. (16856905)
2006
23
Hairy cell leukemia: towards a curative strategy. (16990114)
2006
24
Hairy cell leukemia: clinical presentation and long term follow up after treatment with 2-chlorodeoxyadenosine (2-CdA). (15960289)
2005
25
Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA): long-term follow-up of the Northwestern University experience. (15761021)
2005
26
Expression and activity of NOX5 in the circulating malignant B cells of hairy cell leukemia. (16339585)
2005
27
Hairy cell leukemia: at the crossroad of somatic mutation and isotype switch. (15284115)
2004
28
Successful treatment of a patient with hairy cell leukemia and pentostatin-induced autoimmune thrombocytopenia with rituximab. (12701118)
2003
29
CD52 expression in hairy cell leukemia. (14635201)
2003
30
Treatment of hairy-cell leukemia. (11794207)
2001
31
Efficacy of anti-CD20 monoclonal antibodies (Mabthera) in patients with progressed hairy cell leukemia. (11602410)
2001
32
Malignancy: Current Clinical Practice: Treatment of Hairy Cell Leukemia at the Close of the 20th Century. (11399570)
1999
33
Elevated antibody levels to Epstein-Bbarr virus antigens in patients with hairy cell leukemia compared to controls in relation to exposure to pesticides, organic solvents, animals, and exhausts. (10905566)
1999
34
T-cell dysfunction in hairy cell leukemia: an updated review. (9669674)
1998
35
The hairy cell leukemia cell line Eskol spontaneously synthesizes tumor necrosis factor-alpha and nitric oxide. (9678716)
1998
36
Intermittent interferon alfa in the treatment of hairy cell leukemia]. (9830322)
1998
37
TCR gamma delta+ cells expressing Vgamma 9V delta2, which normally predominate the blood, are found in the spleens of patients with hairy cell leukemia. (9001424)
1997
38
An ultrastructural study of hairy cells of hairy-cell leukemia. (9130401)
1997
39
The development of congestive cardiac failure in a patient with hairy cell leukemia treated with 2-chlorodeoxyadenosine. (9322907)
1997
40
Long-lasting complete remission in patients with hairy cell leukemia treated with 2-CdA: a 5-year survey. (9180283)
1997
41
Molecular study of hairy cell leukemia variant with biclonal paraproteinemia. (9126043)
1997
42
Involvement of the CCND1 gene in hairy cell leukemia. (8740788)
1996
43
Hairy cell leukemia in association with thrombotic thrombocytopenic purpura and factor VIII antibodies. (8819086)
1996
44
Deoxycoformycin induces long-lasting remissions in hairy cell leukemia: clinical and biological results of two different regimens. (7820042)
1994
45
Hairy cells from hairy cell leukemia patients presenting with pronounced polyclonal hypergammaglobulinemia secrete a factor enhancing IgG synthesis. (8432045)
1993
46
Interferon-alpha downregulates the abnormal intracytoplasmic free calcium concentration of tumor cells in hairy cell leukemia. (1382718)
1992
47
Establishment and characterization of an Epstein-Barr virus spontaneously transformed lymphocytic cell line derived from a hairy cell leukemia patient. (1851909)
1991
48
Fludarabine phosphate in refractory hairy cell leukemia. (1708945)
1991
49
Serum soluble interleukin-2 receptor is associated with clinical and pathologic disease status in hairy cell leukemia. (1700727)
1990
50
Combined effects of interferon and 2'-deoxycoformycin on 2',5'-oligoadenylate synthetase and adenosine deaminase in hairy cell and chronic lymphocytic leukemia cells. (2273300)
1990

Genetic Variations for Hairy Cell Leukemia

Expression for genes affiliated with Hairy Cell Leukemia

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
38NCBI BioSystems Database, 30KEGG, 12EMD Millipore, 54Reactome, 52QIAGEN
See all sources

Pathways related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.4IL2RB, BRAF, IL2RG
210.4IL2RG, AICDA, ADA
3
Hide members
10.4IL2RG, IL2RB, TBX21
4
Hide members
10.3IL3RA, IL2RG, IL2RB
5
Immune response IL-2 activation and signaling pathway
Hide members
10.3IL3RA, IL2RG, IL2RB
6
Hide members
10.3IL3RA, IL2RG, IFNA1
7
Hide members
10.3IL2RG, IL2RB, IFNA1, IFNA2
8
Hide members
10.3ITGAX, IL3RA, IL2RG, IL2RB
9
Hide members
10.3IGHM, IL2RG, BRAF, IFNA2, IFNA1
10
Hide members
10.3IL3RA, IL2RG, IL2RB, IFNA1, IFNA2
1110.3IFNA2, IFNA1, IL2RB, IL2RG, IL3RA
1210.3IFNA2, IFNA1, IL2RB, IL2RG, IL3RA
13
Hide members
10.2IFNA2, IFNA1, IGHM, IL2RB, IL2RG, IL3RA

Compounds for genes affiliated with Hairy Cell Leukemia

Sources:
45Novoseek, 60Tocris Bioscience, 29IUPHAR, 11DrugBank, 50PharmGKB, 2BitterDB, 24HMDB
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Compounds related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

(show all 38)
idCompoundScoreTop Affiliating Genes
1pegasys4510.8IFNA1, IFNA2
2entecavir4510.8IFNA2, IFNA1
3pentostatin45 60 29 1113.8IFNA1, IFNA2, ADA
4mizoribine4510.8IFNA2, IFNA1
5denileukin diftitox45 50 1112.8IL2RB, IFNA1, IL2RG
6didanosine45 1111.8IFNA2, IFNA1, ADA
7famciclovir45 1111.8IFNA2, IFNA1
8busulfan45 1111.8ADA, IFNA2, IFNA1
9intron a4510.8IFNA2, IFNA1
10levamisole45 1111.8IFNA1, IFNA2, IL2RB
11n-phosphonacetyl-l-aspartate4510.8IFNA1, IFNA2
122,5-oligoadenylate4510.7IFNA1, ADA, IL2RB, IFNA2
13neopterin4510.7IFNA1, IL2RB, ADA, IFNA2
14polyethylene glycol4510.7ACP5, IFNA1, IFNA2, ADA
15Aldesleukin1110.7IL2RB, IL2RG
16vidarabine45 1111.7ADA, IFNA2
17ribavirin45 50 1112.7IFNA2, ADA, IFNA1
18anagrelide45 1111.7IFNA2, IFNA1
19ifn-alpha4510.7IFNA2, IL2RB, IFNA1
20lomustine45 1111.7IFNA1, IFNA2
215-deoxy-5-fluorouridine4510.7IFNA1, IFNA2
22cyclosporin a45 29 6012.7IL2RB, ADA, ACP5, IL2RG, IFNA1
23aspartate4510.7ADA, ACP5, IL2RB, IFNA1, IFNA2, BRAF
24adefovir4510.6IFNA2, IFNA1
2512-o-tetradecanoylphorbol 13-acetate4510.6IL3RA, IL2RB, IFNA2, BRAF
26cycloheximide4510.6RGS1, IFNA1, ITGAX, IL2RB
27zidovudine45 1111.6IFNA2, ADA, IFNA1
28azathioprine45 50 2 1113.6ACP5, IL2RB, IFNA2
29leukotriene b445 60 2412.5ACP5, IL2RB, ITGAX
30amantadine45 1111.5IFNA1, IFNA2
31retinoic acid45 2411.5IFNA1, BRAF, ITGAX, IL2RB, IFNA2, ADA
32tyrosine4510.5IFNA1, IL2RB, ACP5, IL2RG, IL3RA, C6orf25
33rapamycin4510.5IL2RB, IFNA1, ACP5, BRAF
34cytarabine45 50 1112.4ADA, IFNA2, IFNA1
35glycyrrhizin45 2911.4IFNA1, IL2RB
36vincristine45 50 1112.4ADA, IFNA1, IFNA2
37nucleoside4510.3IFNA1, ADA, IFNA2
38prednisolone45 29 1112.1IFNA2, IL2RB, IFNA1

GO Terms for genes affiliated with Hairy Cell Leukemia

Sources:
16Gene Ontology
See all sources

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

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:00989710.3ADA, IL2RG, IL2RB, ITGAX
2plasma membraneGO:0058869.7ADA, BRAF, ITGAX, C6orf25, PRAME, IGHM

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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Hairy Cell Leukemia

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet