HCL
MCID: HRY003
MIFTS: 57

Hairy Cell Leukemia (HCL) malady

Rare diseases, Cancer diseases, Blood diseases categories

Summaries for Hairy Cell Leukemia

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Sources:
44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 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 is Primary immunodeficiency. The drugs interferon alfa-2a and interferon alfa-2b and the compounds vidarabine and pentostatin have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and bone marrow, and related mouse phenotypes are tumorigenesis and hematopoietic system.

Wikipedia:66 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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9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 46Novoseek, 50Orphanet, 63UMLS, 41NCIt, 28ICD9CM, 59SNOMED-CT, 36MeSH, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet, 26ICD10
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Classifications:

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


Characteristics (Orphanet epidemiological data):

50
hairy cell leukemia:
Prevalence: 1-9/100000; Age of onset: Adulthood


Aliases & Descriptions:

hairy cell leukemia 9 44 11 50 63
leukemic reticuloendotheliosis 44 50
hcl 44 50
leukemia hairy cell 46


External Ids:

Disease Ontology9 DOID:285
NCIt41 C7402
ICD9CM28 202.4
MeSH36 D007943
MESH via Orphanet37 D007943
ICD10 via Orphanet27 C91.4
SNOMED-CT via Orphanet60 118613001, 54087003
UMLS via Orphanet64 C0023443
ICD1026 C91.4

Related Diseases for Hairy Cell Leukemia

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18GeneCards, 19GeneDecks
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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 188)
idRelated DiseaseScoreTop Affiliating Genes
1leukemia32.2CD52, ACP5, IFNA2, ADA, AICDA
2chronic lymphocytic leukemia31.3IGHV4-34
3b-cell lymphomas30.9ACP5, IGHM, AICDA
4hemolytic anemia30.6ADA, IFNA2
5non-hodgkin lymphoma30.6CD52, ADA, AICDA
6b-cell chronic lymphocytic leukemia30.6IGHM, ACP5
7myeloma30.5IGHM, IFNA2, ACP5
8cryoglobulinemia30.4IFNA2
9cutaneous t cell lymphoma30.3ADA, IFNA2
10burkitt's lymphoma30.1AICDA, IGHM, ADA, ACP5, CD52
11hepatitis c29.8AICDA, IGHM, IFNA2
12refractory hairy cell leukemia10.8
13myeloid leukemia10.6
14intrapelvic lymph node leukemic reticuloendotheliosis10.6
15prolymphocytic leukemia10.5
16acute myeloid leukemia10.5
17vasculitis10.5
18hcl-v10.5
19sarcoma10.5
20splenomegaly10.5
21polycythemia vera10.4
22autoimmune hemolytic anemia10.4
23mantle cell lymphoma10.4
24meningitis10.4
25myelofibrosis10.4
26polycythemia10.4
27hyperparathyroidism10.4
28lymphoid leukemia10.3
29polyclonal hypergammaglobulinemia10.3
30splenic manifestation of hairy cell leukemia10.3
31chronic myeloid leukemia10.3
32hairy cell leukemia of spleen10.3
33macroglobulinemia10.3
34splenic marginal zone lymphoma10.3
35sporotrichosis10.3
36aplastic anemia10.3
37dendritic cell sarcoma10.3
38guillain-barre syndrome10.3
39hepatitis10.3
40hodgkin's lymphoma10.3
41marginal zone b-cell lymphoma10.3
42osteosclerosis10.3
43retinitis10.3
44sideroblastic anemia10.3
45essential thrombocythemia10.3
46large granular lymphocyte leukemia10.3
47mycobacterium kansasii10.3
48langerhans cell sarcoma10.2
49melkersson-rosenthal syndrome10.2
50langerhans-cell histiocytosis10.2

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



Diseases related to hairy cell leukemia

Symptoms for Hairy Cell Leukemia

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Drugs & Therapeutics for Hairy Cell Leukemia

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-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 UMLS63/NDF-RT42:

Genetic Tests for Hairy Cell Leukemia

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Anatomical Context for Hairy Cell Leukemia

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34MalaCards
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MalaCards organs/tissues related to Hairy Cell Leukemia:

34
B cells, Bone, Bone marrow, T cells, Spleen, Myeloid, Skin, Monocytes, Colon, Lymph node, Prostate, Pancreas, Kidney, Cortex, Brain, Liver

Animal Models for Hairy Cell Leukemia or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Hairy Cell Leukemia:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.1TBX21, BRAF, IGHM, AICDA
2MP:00053978.4TBX21, ACP5, ADA, BRAF, IGHM, AICDA
3MP:00053878.3AICDA, IGHM, BRAF, ADA, ACP5, TBX21
4MP:00028737.9AICDA, IGHM, BRAF, ADA, CD52

Publications for Hairy Cell Leukemia

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53PubMed
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Articles related to Hairy Cell Leukemia:

(show top 50)    (show all 608)
idTitleAuthorsYear
1
Clinical characteristics and long-term outcome of young hairy cell leukemia patients treated with cladribine: a single-institution series. (24192579)
2014
2
Hairy cell leukemia masquerading as infective endocarditis. (24426343)
2013
3
Hairy Cell Leukemia and cutaneous involvement. (22370038)
2012
4
Treatment of hairy cell leukemia: long-term results in a developing country. (22664112)
2012
5
Application of a BRAF V600E mutation-specific antibody for the diagnosis of hairy cell leukemia. (22531170)
2012
6
Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital. (22499303)
2012
7
Simple genetic diagnosis of hairy cell leukemia by sensitive detection of the BRAF-V600E mutation. (22028477)
2012
8
Variables in the quantification of CD4 in normals and hairy cell leukemia patients. (20687201)
2011
9
Hairy cell leukemia: epidemiology, pharmacokinetics of cladribine, and long-term follow-up of subcutaneous therapy. (21599605)
2011
10
Specific skin lesions in hairy cell leukemia at presentation: case report and review of literature. (19533422)
2010
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: evaluation of the long-term outcome in 121 patients. (20597132)
2010
13
Fenretinide-associated multilayered retinal hemorrhage in a patient with hairy cell leukemia. (21117610)
2010
14
Achievement of complete remission of refractory hairy cell leukemia by rituximab progressing after allogeneic hematopoietic stem cell transplantation. (19330554)
2009
15
IG genes and hairy cell leukemia. (19965713)
2009
16
Hairy cell leukemia. (17673807)
2007
17
Central role of protein kinase Cepsilon in constitutive activation of ERK1/2 and Rac1 in the malignant cells of hairy cell leukemia. (17255340)
2007
18
Acute vasculitis as a first manifestation of hairy cell leukemia. (17449398)
2007
19
Malignant thymoma associated with fatal myocarditis and polymyositis in a 32-year-old woman with a history of hairy cell leukemia. (16601447)
2006
20
Characterization of T-cell repertoire in hairy cell leukemia patients before and after recombinant immunotoxin BL22 therapy. (16311729)
2006
21
V(H) gene analysis of hairy cell leukemia reveals a homogeneous mutation status and suggests its marginal zone B-cell origin. (15356640)
2004
22
Hairy cell leukemia: at the crossroad of somatic mutation and isotype switch. (15284115)
2004
23
Epstein-Barr virus-associated B-cell non-Hodgkin lymphoma following treatment of hairy cell leukemia with cladribine. (14568909)
2003
24
Rituximab: a useful drug for a repeatedly relapsed hairy cell leukemia patient. (12483372)
2002
25
The natural history and clinico-pathological features of the variant form of hairy cell leukemia. (11243388)
2001
26
Atypical mycobacterial infection in a patient with hairy cell leukemia. (11270299)
2001
27
Chromosomal gains and losses are uncommon in hairy cell leukemia: a study based on comparative genomic hybridization and interphase fluorescence in situ hybridization. (11463458)
2001
28
Deoxycoformycin in the treatment of patients with hairy cell leukemia: results of a Spanish collaborative study of 80 patients. (10640967)
2000
29
Malignancy: Current Clinical Practice: Treatment of Hairy Cell Leukemia at the Close of the 20th Century. (11399570)
1999
30
Remission of psoriasis after treatment with interferon-alfa and 2-chlordeoxyadenosine for hairy cell leukemia. (10426918)
1999
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
Development of essential thrombocythemia in a patient treated with interferon alfa and pentostatin for hairy cell leukemia. (9517515)
1998
33
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
34
Correction of abnormal T-cell receptor repertoire during interferon-alpha therapy in patients with hairy cell leukemia. (9596670)
1998
35
Hairy cell leukemia-variant treated with 2-chlorodeoxyadenosine--a report of three cases. (9168448)
1997
36
Hairy cell leukemia is infrequent in MAcxico and has a geographic distribution. (8701952)
1996
37
Involvement of the CCND1 gene in hairy cell leukemia. (8740788)
1996
38
Analysis of response to interferon alpha-2b and the significance of antinuclear antibodies in hairy cell leukemia]. (8656758)
1996
39
Early age onset of hairy cell leukemia presenting with leukocytosis. (8923794)
1996
40
Hairy cell leukemia without splenomegaly nor myelofibrosis in a patient with gastric adenocarcinoma: early phase of the disease or a variant? (7567177)
1995
41
Leukemic cells in hairy cell leukemia and B cell chronic lymphocytic leukemia release soluble TNF receptors. (7596168)
1995
42
Interferon alpha and intracytoplasmic free calcium in hairy cell leukemia cells. (8180601)
1994
43
Interleukin-6 functions as an intracellular growth factor in hairy cell leukemia in vitro. (8227350)
1993
44
A single cycle of 2-chlorodeoxyadenosine results in complete remission in the majority of patients with hairy cell leukemia. (1358262)
1992
45
Serum monocyte colony-stimulating factor, erythropoietin and interleukin-6 in relation to pancytopenia in hairy cell leukemia. (1625496)
1992
46
Hairy cell leukemia: splenectomy after alpha 2b-interferon therapy. (1878598)
1991
47
Heterogeneous nuclear RNA from hairy cell leukemia patients activates 2',5'-oligoadenylate synthetase. (1768064)
1991
48
Treatment of hairy cell leukemia with alternating cycles of pentostatin and recombinant leukocyte A interferon: results of a phase II study. (2313337)
1990
49
Hairy cell leukemia with chylous ascites. (578227)
1977
50
Hairy cell leukemia ('leukemic reticuleondotheliosis'), reticulosarcoma, and monocytic leukemia. Cytochemical and ultrastructural investigations. (808067)
1975

Variations for Hairy Cell Leukemia

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Expression for genes affiliated with Hairy Cell Leukemia

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2BioGPS, 16Gene 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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51PathCards, 31KEGG
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Pathways related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6ADA, AICDA

Compounds for genes affiliated with Hairy Cell Leukemia

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46Novoseek, 12DrugBank, 62Tocris Bioscience, 30IUPHAR, 52PharmGKB, 25HMDB
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Compounds related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

(show all 14)
idCompoundScoreTop Affiliating Genes
1vidarabine46 1211.0IFNA2, ADA
2pentostatin46 62 30 1213.0IFNA2, ADA
3didanosine46 52 1211.9ADA, IFNA2
4busulfan46 52 1211.9IFNA2, ADA
5allopurinol46 52 1211.9IFNA2, ADA
6inosine46 2510.8IFNA2, ADA
72,5-oligoadenylate469.7IFNA2, ADA
8ribavirin46 52 1211.7IFNA2, ADA
9polyethylene glycol469.7ACP5, IFNA2, ADA
10deoxycytidine46 2510.7AICDA, ADA
11cytidine46 25 1211.5ADA, AICDA
12aspartate469.4ACP5, IFNA2, ADA, BRAF
13estrogen469.0AICDA, BRAF, ADA, IFNA2, ACP5
14fludarabine46 52 1210.9CD52, ADA

GO Terms for genes affiliated with Hairy Cell Leukemia

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17Gene Ontology
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Biological processes related to Hairy Cell Leukemia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of inflammatory responseGO:0507289.7ACP5, ADA

Products for genes affiliated with Hairy Cell Leukemia

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Sources for Hairy Cell Leukemia

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet