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, 48Orphanet, 60UMLS, 44Novoseek, 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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Sources:
32MalaCards
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MalaCards organs/tissues related to Hairy Cell Leukemia:

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

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
A rare occurrence of hairy cell leukemia in a congolese child: a presentation and challenge of diagnosis in low resource settings. (23652869)
2013
2
Disseminated tuberculosis mimicking relapse in hairy cell leukemia. (24369208)
2013
3
Killing two birds with one stone: BRAF V600E inhibitor therapy for hairy cell leukemia and Langerhans/dendritic cell sarcoma. (23322213)
2013
4
Treatment of hairy cell leukemia: long-term results in a developing country. (22664112)
2012
5
Langerhans/dendritic cell sarcoma arising from hairy cell leukemia: a rare phenomenon. (22234838)
2012
6
The BRAF V600E mutation in hairy cell leukemia and other mature B-cell neoplasms. (22072557)
2012
7
Hairy cell leukemia with ascites: an unusual presentation. (20578814)
2011
8
Diagnosis of hairy cell leukemia by flow cytometry. (21504292)
2011
9
Hairy cell leukemia variant, a new entity of the WHO 2008. (22105821)
2011
10
Higher expression levels of activation-induced cytidine deaminase distinguish hairy cell leukemia from hairy cell leukemia-variant and splenic marginal zone lymphoma. (20237507)
2010
11
Sustained long-term remissions with weekly interferon maintenance therapy in hairy cell leukemia. (20887503)
2010
12
Cost-effectiveness of pentostatin compared with cladribine in the management of hairy cell leukemia in the United Kingdom. (20110049)
2009
13
Long-term follow-up studies in hairy cell leukemia. (19814694)
2009
14
Effective treatment of a refractory hairy cell leukemia variant with splenic pre-irradiation and alemtuzumab. (18259114)
2008
15
T-bet transcription factor detection facilitates the diagnosis of minimal hairy cell leukemia infiltrates in bone marrow trephines. (17667540)
2007
16
Malignant thymoma associated with fatal myocarditis and polymyositis in a 32-year-old woman with a history of hairy cell leukemia. (16601447)
2006
17
Pentostatin: impact on outcome in hairy cell leukemia. (16990110)
2006
18
Development of hairy cell leukemia in a patient after cardiac transplantation. (16321873)
2006
19
Hairy cell leukemia. (16953542)
2006
20
Hairy cell leukemia: survival and relapse. Long-term follow-up of purine analog-based therapy and approach for relapsed disease. (15737878)
2005
21
Two new cases of familial hairy cell leukemia associated with HLA haplotypes A2, B7, Bw4, Bw6. (15621808)
2005
22
An update: 12-year follow-up of patients with hairy cell leukemia following treatment with 2-chlorodeoxyadenosine. (15229616)
2004
23
Bilateral facial nerve palsy as first indication of relapsing hairy cell leukemia after 36 years. (15277655)
2004
24
Interferon in the treatment of hairy-cell leukemia. (12670466)
2003
25
Extended follow-up of patients with hairy cell leukemia after treatment with cladribine. (12610190)
2003
26
CD11c gene expression in hairy cell leukemia is dependent upon activation of the proto-oncogenes ras and junD. (12576324)
2003
27
Cladribine (2-chlorodeoxyadenosine) therapy in hairy cell leukemia variant. A report of three cases. (11801472)
2002
28
Hairy cell leukemia: biology, clinical diagnosis, unusual manifestations and associated disorders. (12823778)
2002
29
The natural history and clinico-pathological features of the variant form of hairy cell leukemia. (11243388)
2001
30
Hairy cell leukemia-variant. (11255616)
2001
31
Reconstitution of endogenous interferon a by recombinant interferon in hairy cell leukemia. (11034083)
2000
32
2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia and hairy cell leukemia variant: 7-year experience in Poland. (9918312)
1999
33
Responses in refractory hairy cell leukemia to a recombinant immunotoxin. (10552943)
1999
34
How reliable is histologic examination of bone marrow trephine biopsy specimens for the staging of non-Hodgkin lymphoma? A study of hairy cell leukemia and mantle cell lymphoma involvement of the bone marrow trephine specimen by histologic, immunohistochemical, and polymerase chain reaction techniques. (9930138)
1999
35
Accumulating mutations of p53 in colon tumor and hairy cell leukemia do not arise from methylation/deamination processes, but rather from nucleotide deletions and insertions. (9628350)
1998
36
Familial hairy cell leukemia: a HLA-linked disease or farmers-linked disease? (9793263)
1998
37
Blastic transformation of hairy cell leukemia. (9240906)
1997
38
Treatment of hairy-cell leukemia with cladribine: response, toxicity, and long-term follow-up. (9060556)
1997
39
Remission in hairy cell leukemia-variant following splenic radiotherapy alone. (9322903)
1997
40
Workshop on chronic lymphocytic leukemia and hairy cell leukemia. Results of a consensus conference of the German CLL Cooperative Group. Peer-reviewed proceedings. Berlin, Germany, February 7-10, 1996. (9213634)
1997
41
Effects of interferon-alpha on L-BCGF- and TNF-alpha-induced proliferation of hairy cell leukemia in Japan. (7845025)
1995
42
Use of 2-chlorodeoxyadenosine, granulocyte-colony-stimulating factor, and erythropoietin in a Jehovah's Witness with hairy cell leukemia. (7541605)
1995
43
Impaired interferon alpha response in hairy cell leukemia is corrected by therapy with 2-chloro-2'-deoxyadenosine: implications for susceptibility to opportunistic infections. (7916389)
1994
44
Natural-killer-stimulatory effect of combined low-dose interleukin-2 and interferon beta in hairy-cell leukemia patients. (7512888)
1994
45
The therapeutic effect of interferon-alpha exemplified by hairy cell leukemia]. (8273363)
1993
46
Serum monocyte colony-stimulating factor, erythropoietin and interleukin-6 in relation to pancytopenia in hairy cell leukemia. (1625496)
1992
47
Heterogeneous nuclear RNA from hairy cell leukemia patients activates 2',5'-oligoadenylate synthetase. (1768064)
1991
48
Loss of interferon antibodies during prolonged continuous interferon-alpha 2a therapy in hairy cell leukemia. (1704264)
1991
49
Production and secretion of BSF2/IL6 in a case of hairy cell leukemia with polyclonal hypergammaglobulinemia]. (2386010)
1990
50
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with hairy-cell leukemia. (6625120)
1983

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
Hide members
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:00588610.0BRAF, ITGAX, PRAME, IGHM, IL2RB, IL2RG
2external side of plasma membraneGO:00989710.0ITGAX, IL2RB, IL2RG, ADA

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