HCL
MCID: HRY003
MIFTS: 59

Hairy Cell Leukemia (HCL) malady

Rare diseases, Cancer diseases, Blood diseases categories
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Summaries for Hairy Cell Leukemia

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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 based summary: 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:65 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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Sources:
8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 48Orphanet, 62UMLS, 57SNOMED-CT, 34MeSH, 39NCIt, 27ICD9CM, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet, 25ICD10
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Hairy Cell Leukemia, Aliases & Descriptions:

Name: Hairy Cell Leukemia 8 42 10 48 62
Leukemic Reticuloendotheliosis 42 48
Hcl 42 48
 
Leukaemic Reticuloendotheliosis 62
Leukemia Hairy Cell 44


Classifications:

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


Characteristics (Orphanet epidemiological data):

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


External Ids:

Disease Ontology8 DOID:285
MeSH34 D007943
NCIt39 C7402
ICD9CM27 202.4
MESH via Orphanet35 D007943
ICD10 via Orphanet26 C91.4
UMLS via Orphanet63 C0023443
ICD1025 C91.4

Related Diseases for Hairy Cell Leukemia

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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
1leukemia32.0CD52, ACP5, IFNA2, ADA, AICDA
2chronic lymphocytic leukemia31.5ACP5, IGHM
3cryoglobulinemia31.0IFNA2
4b-cell lymphomas31.0AICDA, ACP5, IGHM
5hemolytic anemia30.8ADA, IFNA2
6myeloma30.7ACP5, IFNA2, IGHM
7non-hodgkin lymphoma30.7ADA, CD52, AICDA
8cutaneous t cell lymphoma30.4ADA, IFNA2
9hepatitis c29.9IFNA2, IGHM, AICDA
10burkitt's lymphoma29.7AICDA, IGHM, ADA, ACP5, CD52
11refractory hairy cell leukemia10.8
12myeloid leukemia10.6
13intrapelvic lymph node leukemic reticuloendotheliosis10.6
14hcl-v10.6
15prolymphocytic leukemia10.6
16acute myeloid leukemia10.5
17sarcoma10.5
18vasculitis10.5
19hyperparathyroidism10.4
20mantle cell lymphoma10.4
21autoimmune hemolytic anemia10.4
22splenic manifestation of hairy cell leukemia10.4
23polycythemia vera10.4
24meningitis10.4
25polycythemia10.4
26splenomegaly10.4
27aplastic anemia10.3
28chronic myeloid leukemia10.3
29hepatitis10.3
30hodgkin's lymphoma10.3
31lymphoid leukemia10.3
32marginal zone b-cell lymphoma10.3
33myelofibrosis10.3
34splenic marginal zone lymphoma10.3
35polyclonal hypergammaglobulinemia10.3
36hairy cell leukemia of spleen10.3
37macroglobulinemia10.3
38dendritic cell sarcoma10.3
39guillain-barre syndrome10.3
40osteosclerosis10.3
41retinitis10.3
42acute febrile neutrophilic dermatosis10.3
43essential thrombocythemia10.3
44large granular lymphocyte leukemia10.3
45mycobacterium kansasii10.3
46cryoglobulinemic vasculitis10.3IGHM, IFNA2
47leukopenia10.3IFNA2, CD52
48myocardial infarction10.2
49gaucher's disease10.2ADA, ACP5
50glomerulonephritis10.2

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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Genetic Tests for Hairy Cell Leukemia

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Anatomical Context for Hairy Cell Leukemia

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MalaCards organs/tissues related to Hairy Cell Leukemia:

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

Animal Models for Hairy Cell Leukemia or affiliated genes

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MGI Mouse Phenotypes related to Hairy Cell Leukemia:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.1TBX21, BRAF, IGHM, AICDA
2MP:00053978.4TBX21, AICDA, IGHM, BRAF, ADA, ACP5
3MP:00053878.3IGHM, BRAF, ADA, ACP5, TBX21, AICDA
4MP:00028737.9CD52, AICDA, IGHM, BRAF, ADA

Publications for Hairy Cell Leukemia

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Articles related to Hairy Cell Leukemia:

(show top 50)    (show all 629)
idTitleAuthorsYear
1
Hematopoietic stem cell origin of BRAFV600E mutations in hairy cell leukemia. (24871132)
2014
2
Autoimmune disorders in patients with hairy cell leukemia: are they more common than previously thought? (25265129)
2014
3
Clinical characteristics and long-term outcome of young hairy cell leukemia patients treated with cladribine: a single-institution series. (24192579)
2014
4
High prevalence of MAP2K1 mutations in variant and IGHV4-34-expressing hairy-cell leukemias. (24241536)
2014
5
Hairy cell leukemia masquerading as infective endocarditis. (24426343)
2013
6
Hairy Cell Leukemia and cutaneous involvement. (22370038)
2012
7
Treatment of hairy cell leukemia: long-term results in a developing country. (22664112)
2012
8
Application of a BRAF V600E mutation-specific antibody for the diagnosis of hairy cell leukemia. (22531170)
2012
9
Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital. (22499303)
2012
10
Simple genetic diagnosis of hairy cell leukemia by sensitive detection of the BRAF-V600E mutation. (22028477)
2012
11
Variables in the quantification of CD4 in normals and hairy cell leukemia patients. (20687201)
2011
12
Hairy cell leukemia: epidemiology, pharmacokinetics of cladribine, and long-term follow-up of subcutaneous therapy. (21599605)
2011
13
Specific skin lesions in hairy cell leukemia at presentation: case report and review of literature. (19533422)
2010
14
Very long-term eradication of minimal residual disease in patients with hairy cell leukemia after a single course of cladribine. (20056789)
2010
15
Hairy cell leukemia: evaluation of the long-term outcome in 121 patients. (20597132)
2010
16
Fenretinide-associated multilayered retinal hemorrhage in a patient with hairy cell leukemia. (21117610)
2010
17
Achievement of complete remission of refractory hairy cell leukemia by rituximab progressing after allogeneic hematopoietic stem cell transplantation. (19330554)
2009
18
IG genes and hairy cell leukemia. (19965713)
2009
19
Hairy cell leukemia. (17673807)
2007
20
Central role of protein kinase Cepsilon in constitutive activation of ERK1/2 and Rac1 in the malignant cells of hairy cell leukemia. (17255340)
2007
21
Acute vasculitis as a first manifestation of hairy cell leukemia. (17449398)
2007
22
Malignant thymoma associated with fatal myocarditis and polymyositis in a 32-year-old woman with a history of hairy cell leukemia. (16601447)
2006
23
Characterization of T-cell repertoire in hairy cell leukemia patients before and after recombinant immunotoxin BL22 therapy. (16311729)
2006
24
V(H) gene analysis of hairy cell leukemia reveals a homogeneous mutation status and suggests its marginal zone B-cell origin. (15356640)
2004
25
Hairy cell leukemia: at the crossroad of somatic mutation and isotype switch. (15284115)
2004
26
Rituximab: a useful drug for a repeatedly relapsed hairy cell leukemia patient. (12483372)
2002
27
The natural history and clinico-pathological features of the variant form of hairy cell leukemia. (11243388)
2001
28
Atypical mycobacterial infection in a patient with hairy cell leukemia. (11270299)
2001
29
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
30
Deoxycoformycin in the treatment of patients with hairy cell leukemia: results of a Spanish collaborative study of 80 patients. (10640967)
2000
31
Malignancy: Current Clinical Practice: Treatment of Hairy Cell Leukemia at the Close of the 20th Century. (11399570)
1999
32
Remission of psoriasis after treatment with interferon-alfa and 2-chlordeoxyadenosine for hairy cell leukemia. (10426918)
1999
33
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
34
Development of essential thrombocythemia in a patient treated with interferon alfa and pentostatin for hairy cell leukemia. (9517515)
1998
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
Correction of abnormal T-cell receptor repertoire during interferon-alpha therapy in patients with hairy cell leukemia. (9596670)
1998
37
Hairy cell leukemia-variant treated with 2-chlorodeoxyadenosine--a report of three cases. (9168448)
1997
38
Hairy cell leukemia is infrequent in MAcxico and has a geographic distribution. (8701952)
1996
39
Involvement of the CCND1 gene in hairy cell leukemia. (8740788)
1996
40
Analysis of response to interferon alpha-2b and the significance of antinuclear antibodies in hairy cell leukemia]. (8656758)
1996
41
Early age onset of hairy cell leukemia presenting with leukocytosis. (8923794)
1996
42
Leukemic cells in hairy cell leukemia and B cell chronic lymphocytic leukemia release soluble TNF receptors. (7596168)
1995
43
Interferon alpha and intracytoplasmic free calcium in hairy cell leukemia cells. (8180601)
1994
44
Interleukin-6 functions as an intracellular growth factor in hairy cell leukemia in vitro. (8227350)
1993
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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Expression patterns in normal tissues for genes affiliated with Hairy Cell Leukemia

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Pathways for genes affiliated with Hairy Cell Leukemia

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

(show all 14)
idCompoundScoreTop Affiliating Genes
1vidarabine44 1111.0IFNA2, ADA
2pentostatin44 61 28 1113.0IFNA2, ADA
3didanosine44 50 1111.9ADA, IFNA2
4busulfan44 50 1111.9IFNA2, ADA
5allopurinol44 50 1111.9IFNA2, ADA
6inosine44 2410.8IFNA2, ADA
72,5-oligoadenylate449.7IFNA2, ADA
8ribavirin44 50 1111.7IFNA2, ADA
9polyethylene glycol449.7ACP5, IFNA2, ADA
10deoxycytidine44 2410.7AICDA, ADA
11cytidine44 24 1111.5ADA, AICDA
12aspartate449.4ACP5, IFNA2, ADA, BRAF
13estrogen449.0AICDA, BRAF, ADA, IFNA2, ACP5
14fludarabine44 50 1110.9CD52, ADA

GO Terms for genes affiliated with Hairy Cell Leukemia

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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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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet