DISEASE: Hairy cell leukemia


Entry
H00006                      Disease                                
Name
Hairy cell leukemia
  Supergrp
Non-Hodgkin lymphoma [DS:H02418]
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
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]
Moxetumomab pasudotox [DR:D09932]
Pentostatin [DR:D00155]
Interferon alfa-2b [DR:D02745]
Other DBs
ICD-11: 2A82.2
ICD-10: C91.4
MeSH: D007943
Reference
PMID:12670461
  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

» Japanese version

  All links  
Ontology (1)   
   KEGG BRITE (1)   
Drug (6)   
   KEGG DRUG (6)   
Gene (6)   
   KEGG ORTHOLOGY (3)   
   KEGG GENES (3)   
Literature (1)   
   PubMed (1)   
All databases (14)   

Download RDF