Anaplastic large-cell lymphoma

Definition:

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.

References:

[1]. Greg Hapgood, et al. The biology and management of systemic anaplastic large cell lymphoma. Blood. 2015 Jul 2;126(1):17-25. [Content Brief]

[2]. Joseph Vadakara, et al. Targeting CD30 in anaplastic large cell lymphoma. Curr Hematol Malig Rep. 2012 Dec;7(4):285-91. [Content Brief]

[3]. Philippe Gaulard, et al. ALK-negative anaplastic large-cell lymphoma. Blood. 2016 Jan 14;127(2):175-7. [Content Brief]

[4]. Toby A Eyre, et al. Anaplastic lymphoma kinase-positive anaplastic large cell lymphoma: current and future perspectives in adult and paediatric disease. Eur J Haematol. 2014 Dec;93(6):455-68. [Content Brief]

[5]. Xiaoming Xing, et al. Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous. Adv Anat Pathol. 2015 Jan;22(1):29-49. [Content Brief]

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