Journal
EXPERT REVIEW OF HEMATOLOGY
Volume 9, Issue 2, Pages 137-150Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1586/17474086.2016.1122514
Keywords
Anaplastic large-cell lymphoma; ALK translocation; NPM-ALK fusion; DUSP22 rearrangements; T-cell non-Hodgkin lymphoma; brentuximab vedotin; crizotinib
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Funding
- National Cancer Institute [R01 CA177734]
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Systemic anaplastic large-cell lymphomas (sALCLs) comprise a heterogeneous group of relatively rare T-cell non-Hodgkin lymphomas (NHLs) characterized by CD30 expression and other unifying pathologic features. Anaplastic lymphoma kinase (ALK) fusions are present in about 50% of cases. Pathological diagnosis can be challenging, particularly in ALK-negative cases. Though ALK-positive and ALK-negative sALCLs are similar morphologically and immunophenotypically, they are separate entities with different genetics, clinical behavior, and outcomes. Evidence-based data evaluating treatment regimens are limited as randomized controlled trials are lacking and most prospective studies are too small to draw definitive conclusions. However, recent advances in molecular biology are bringing forth much-needed knowledge in this field, and are likely to guide further targeted therapeutic development.
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