KEGG   DISEASE: Hairy cell leukemiaHelp
Entry
H00006                      Disease                                

Name
Hairy cell leukemia
Description
Hairy cell Leukemia (HCL) is a chronic lymphoproliferative disorder that is defined, according to the WHO classification, as a mature (peripheral) B-cell neoplasm. HCL accounts for between 2-3% of all leukemia cases, with about 600 new cases diagnosed in the U.S. each year. With regard to genetic abnormalities of potential pathogenetic importance in HCL, only a few have been described. Mutations of p53 and Bcl-6 have been reported in about one-third of cases, but their functional significance is unclear. Over-expression of cyclin D1, an important cell cycle regulator, has been reported in cases of HCL as well as mantle cell lymphoma (MCL) and in a few cases of chronic lymphocytic leukemia among lymphoid malignancies.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00006  Hairy cell leukemia
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature B-cell neoplasms
    2A82  Mature B-cell neoplasm with leukaemic behaviour
     H00006  Hairy cell leukemia
Tumor markers [br08442.html]
 H00006
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Gene
p53 (mutation) [HSA:7157] [KO:K04451]
Bcl-6 (mutation) [HSA:604] [KO:K15618]
Cyclin D1 (overexpression) [HSA:595] [KO:K04503]
Drug
Cladribine [DR:D01370]
Rituximab [DR:D02994] (CD20-positive)
Pentostatin [DR:D00155]
Interferon alfa-2b [DR:D02745]
Plerixafor [DR:D08971]
Moxetumomab pasudotox [DR:D09932]
Comment
ICD-O: 9940/3, Tumor type: Hairy cell leukemia
Other DBs
ICD-11: 2A82.2
ICD-10: C91.4
MeSH: D007943
Reference
PMID:12670461 (gene, tumor type)
  Authors
Zuzel M, Cawley JC.
  Title
The biology of hairy cells.
  Journal
Best Pract Res Clin Haematol 16:1-13 (2003)
DOI:10.1016/S1521-6926(02)00082-8
Reference
(drug)
  Authors
Papadakis MA and McPhee SJ |(ed)
  Title
Current Medical Diagnosis & Treatment 2015, Fifty-Fourth Edition
  Journal
McGraw Hill Education
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