|

Variant ALK-fusion positive anaplastic large cell lymphoma (ALCL): A population-based paediatric study of the NHL-BFM study group.

Luedersen, Jette; Stadt, Udo Zur; Richter, Julia; Oschlies, Ilske; Klapper, Wolfram; Rosenwald, Andreas; Kalinova, Marketa; Simonitsch-Klupp, Ingrid; Siebert, Reiner; Zimmermann, Martin; Qi, Minyue; Nakel, Jacqueline; Scheinemann, Katrin; Knörr, Fabian; Attarbaschi, Andishe; Kabickova, Edita; Woessmann, Wilhelm; Damm-Welk, Christine.

Br J Haematol ; 204(5): 1894-1898, 2024 May.

Article En | MEDLINE | ID: mdl-38279625

Frequency, distribution and prognostic meaning of ALK-partner genes other than NPM1 in ALK-positive anaplastic large-cell lymphoma (ALCL) are unknown. Forty-nine of 316 ALCL diagnosed in the NHL-BFM study group showed no nuclear ALK expression suggestive of a variant ALK-partner; 41 were analysed by genomic capture high-throughput sequencing or specific RT-PCRs. NPM1::ALK was detected in 13 cases. Among the 28 patients with a non-NPM1::ALK-fusion partner, ATIC (n = 8; 29%) and TPM3 (n = 9; 32%) were the most common. Five of eight patients with ATIC::ALK-positive ALCL relapsed, none of nine with TPM3::ALK. Variant ALK-partners are rare and potentially associated with different prognoses.

Anaplastic Lymphoma Kinase , Lymphoma, Large-Cell, Anaplastic , Nucleophosmin , Humans , Lymphoma, Large-Cell, Anaplastic/genetics , Lymphoma, Large-Cell, Anaplastic/pathology , Child , Male , Female , Anaplastic Lymphoma Kinase/genetics , Anaplastic Lymphoma Kinase/analysis , Adolescent , Child, Preschool , Oncogene Proteins, Fusion/genetics , Prognosis , Nuclear Proteins/genetics , Nuclear Proteins/metabolism , Infant , Tropomyosin