KEGG   DISEASE: Anaplastic large-cell lymphomaHelp
Entry
H01601                      Disease                                

Name
Anaplastic large-cell lymphoma
  Supergrp
Peripheral T cell lymphoma [DS:H01892]
Description
Anaplastic large cell lymphoma (ALCL) is a lymphoid neoplasm characterized by a proliferation of large lymphoid cells, referred to as hallmark cells, with strong expression of CD30. The World Health Organization recognizes 3 entities: primary cutaneous ALCL (pcALCL), anaplastic lymphoma kinase (ALK)-positive ALCL, and, provisionally, ALK-negative ALCL. pcALCL presents in the skin and, while it may involve locoregional lymph nodes, rarely disseminates. ALK-positive ALCL, by definition overexpresses an ALK-fusion gene, typically via t(2;5)(p23;35) which fuses the ALK gene on chromosome 2 with the nucleophosmin (NPM) gene on chromosome 5, resulting in a NPM-ALK chimeric protein with constitutive tyrosine kinase activity. ALK-negative ALCLs lack ALK rearrangements and their genetic and clinical features are more variable. A subset of ALK-negative ALCLs has rearrangements at 6p25.3 involving DUSP22, a gene encoding a dual-specificity phosphatase that inhibits T-cell receptor signaling. DUSP22 rearrangements also are seen in a subset of pcALCLs.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H01601  Anaplastic large-cell lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature T-cell or NK-cell neoplasms
    2A90  Mature T-cell lymphoma, specified types, nodal or systemic
     H01601  Anaplastic large-cell lymphoma
Tumor markers [br08442.html]
 H01601
BRITE hierarchy
Gene
NPM-ALK (translocation) [HSA:238] [KO:K05119]
DUSP22-IRF4 (translocation) [HSA:56940] [KO:K14165]
Drug
Brentuximab vedotin [DR:D09587]
Comment
ICD-O-3: 9718/3 Primary cutaneous CD30+ T-cell lymphoproliferative disorder (C44._)
ICD-O-3: 9702/3 Mature T-cell lymphoma, NOS
ICD-O-3: 9714/3 Anaplastic large cell lymphoma, T cell and Null cell type
Other DBs
ICD-11: 2A90.A 2A90.B
ICD-10: C84.6 C84.7
MeSH: D017728
Reference
PMID:25869285 (gene, drug)
  Authors
Hapgood G, Savage KJ
  Title
The biology and management of systemic anaplastic large cell lymphoma.
  Journal
Blood 126:17-25 (2015)
DOI:10.1182/blood-2014-10-567461
Reference
PMID:23065407 (drug)
  Authors
Vadakara J, Pro B
  Title
Targeting CD30 in anaplastic large cell lymphoma.
  Journal
Curr Hematol Malig Rep 7:285-91 (2012)
DOI:10.1007/s11899-012-0137-y
Reference
PMID:25461779 (description, gene, ICD-O-3)
  Authors
Xing X, Feldman AL
  Title
Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous.
  Journal
Adv Anat Pathol 22:29-49 (2015)
DOI:10.1097/PAP.0000000000000047
Reference
PMID:24766435 (description, gene, ICD-O-3, drug)
  Authors
Eyre TA, Khan D, Hall GW, Collins GP
  Title
Anaplastic lymphoma kinase-positive anaplastic large cell lymphoma: current and future perspectives in adult and paediatric disease.
  Journal
Eur J Haematol 93:455-68 (2014)
DOI:10.1111/ejh.12360
Reference
  Authors
Gaulard P, de Leval L
  Title
ALK-negative anaplastic large-cell lymphoma.
  Journal
Blood 127:175-7 (2016)
DOI:10.1182/blood-2015-11-676916

» Japanese version

DBGET integrated database retrieval system