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JAK2 rearrangements are a recurrent alteration in CD30+ systemic T-cell lymphomas with anaplastic morphology

American Journal of Surgical Pathology Jun 19, 2021

Fitzpatrick MJ, Massoth LR, Marcus C, et al. - While some peripheral T-cell lymphoma (PTCL) subtypes are well defined by histology, immunophenotype, and recurrent molecular alterations, incomplete information remains concerning other subtypes. In particular, there is disagreement concerning the distinction between CD30+ PTCL, not otherwise specified and anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma. In this study, a series of 6 JAK2 rearrangements occurring in a cohort of 97 CD30+ ALK− PTCL (6%) has been reported, gathered after the detection of an index case of a novel PABPC1-JAK2 fusion in a case of ALK− anaplastic large cell lymphoma with unusual classic Hodgkin lymphoma (CHL)-like features. JAK2 rearrangements were identified in five of six cases (83%) with 4 novel partners: TFG, PABPC1, ILF3, and MAP7, and there was one case demonstrating a previously reported PCM1-JAK2 fusion. Findings overall suggest that JAK2 rearrangements are carried by a subset of CD30+ ALK− systemic PTCL with anaplastic morphology; CHL-like morphologic features are identified in some of these cases. In cases of CD30+ PTCL, the presence of JAK2 rearrangements augments current classification and may present a therapeutic target via JAK2 inhibition.

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