| H01601 | |
| H number | H01601 |
| Name | Anaplastic large-cell lymphoma |
| 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 |
| Network | - |
| Gene | NPM-ALK (translocation) [HSA:238] [KO:K05119] DUSP22-IRF4 (translocation) [HSA:56940] [KO:K14165] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Crizotinib [DR:D09731] (ALK-positive) Brentuximab vedotin [DR:D09587] |
| Comment | - |
| Other DBs | ICD-11: 2A90.A 2A90.B ICD-10: C84.6 C84.7 MeSH: D017728 |
| Reference | PMID:25869285 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 PMID:23065407 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 PMID:25461779 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 PMID:24766435 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 PMID:26769771 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 |