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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.

SUBMITTER: Bennani-Baiti N

PROVIDER: S-EPMC4876810 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature

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Adult systemic anaplastic large-cell lymphoma: recommendations for diagnosis and management.

Bennani-Baiti Nabila N Ansell Stephen S Feldman Andrew L AL

Expert review of hematology 20151211 2

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 differ ...[more]

PMID: 26581318

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Project description:Antibody-based immunotherapy has become an integral part of cancer therapeutics. However, monoclonal antibodies have their limitations as identifying an antigen selectively expressed on malignant cells and developing a high-affinity antibody may not by itself alter tumor growth. This is illustrated in the case of CD30; CD30 epitomizes many properties of an ideal pharmacologic target such as high expression on malignant cells and limited expression on normal tissues. However, until the advent of brentuximab vedotin, CD30 remained an elusive target as antibody-based anti-CD30 immunotherapy had been largely clinically unsuccessful. Brentuximab vedotin (cAC10-vcMMAE, SGN-35) is an antibody-drug conjugate consisting of a chimeric anti-CD30 monoclonal antibody whereupon the potent microtubule inhibitor monomethyl auristatin E (MMAE) is attached via a valine-citrulline linker. Once bound to CD30, brentuximab vedotin is internalized and MMAE is released with the action of lysosomal enzymes on the linker. In phase I studies in relapsed or refractory Hodgkin lymphoma and anaplastic large cell lymphoma, brentuximab vedotin induced unprecedented responses with manageable toxicity. In phase II studies, brentuximab vedotin induced overall response rates of 75% and 86% in relapsed or refractory Hodgkin lymphoma and anaplastic large cell lymphoma, respectively. The results of these trials led to the accelerated approval of the drug by the US Food and Drug Administration in a patient population with few other alternative options. Brentuximab vedotin has overall manageable toxicity profile; however, cumulative peripheral neuropathy constitutes an important clinical consideration as it may limit prolonged administration of the drug. The mechanism by which brentuximab vedotin exerts its antitumor activity is not entirely clear. Diffusion of MMAE in the tumor microenvironment and cytotoxicity on bystander cells may in part explain its activity, especially in Hodgkin lymphoma. Herein, we review the biology of CD30 and brentuximab vedotin, and the clinical data that has accumulated thus far with SGN-35.

Dataset Information

Adult systemic anaplastic large-cell lymphoma: recommendations for diagnosis and management.

Publications

Adult systemic anaplastic large-cell lymphoma: recommendations for diagnosis and management.

Similar Datasets

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