title: Adult systemic anaplastic large-cell lymphoma: recommendations for diagnosis and management.
abstract: 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.
authors.affiliation: a Division of Hematology , Mayo Clinic , Rochester , MN , USA.
authors.forename: Nabila
authors.initials: N
authors.lastname: Bennani-Baiti
authors.affiliation: a Division of Hematology , Mayo Clinic , Rochester , MN , USA.
authors.forename: Stephen
authors.initials: S
authors.lastname: Ansell
authors.affiliation: b Department of Laboratory Medicine and Pathology , Mayo Clinic , Rochester , MN , USA.
authors.forename: Andrew L
authors.initials: AL
authors.lastname: Feldman
mesh_terms: D000328:Adult; D000077548:Anaplastic Lymphoma Kinase; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D056915:DNA Copy Number Variations; D019468:Disease Management; D019008:Drug Resistance, Neoplasm; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016130:Immunophenotyping; D017728:Lymphoma, Large-Cell, Anaplastic / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D009367:Neoplasm Staging; D011379:Prognosis; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics / Q000378:metabolism; D012008:Recurrence; D012074:Remission Induction; D014178:Translocation, Genetic; D014182:Transplantation, Autologous
chemical_list: C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases
keywords: ALK translocation; Anaplastic large-cell lymphoma; DUSP22 rearrangements; NPM-ALK fusion; T-cell non-Hodgkin lymphoma; brentuximab vedotin; crizotinib