TY - JOUR
T1 - Adult systemic anaplastic large-cell lymphoma
T2 - Recommendations for diagnosis and management
AU - Bennani-Baiti, Nabila
AU - Ansell, Stephen
AU - Feldman, Andrew L.
N1 - Publisher Copyright:
© 2015 Taylor and Francis.
PY - 2016/2/1
Y1 - 2016/2/1
N2 - 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.
AB - 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.
KW - ALK translocation
KW - Anaplastic large-cell lymphoma
KW - DUSP22 rearrangements
KW - NPM-ALK fusion
KW - T-cell non-Hodgkin lymphoma
KW - brentuximab vedotin
KW - crizotinib
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U2 - 10.1586/17474086.2016.1122514
DO - 10.1586/17474086.2016.1122514
M3 - Review article
C2 - 26581318
AN - SCOPUS:84957845280
SN - 1747-4086
VL - 9
SP - 137
EP - 150
JO - Expert Review of Hematology
JF - Expert Review of Hematology
IS - 2
ER -