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Published on: August 23, 2024
Anaplastic large cell lymphoma, ALK-positive.
Andrés J M Ferreri1, Silvia Govi, Stefano A Pileri
1Unit of Lymphoid Malignancies, San Raffaele Scientific Institute, Milan, Italy. andres.ferreri@hsr.it
Critical Reviews in Oncology/Hematology
|March 24, 2012
Summary
Anaplastic lymphoma kinase-positive (ALK+) anaplastic large cell lymphoma (ALCL) is an aggressive T-cell lymphoma. Standard chemotherapy offers good outcomes, with novel therapies showing promise for relapsed or refractory cases.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- Anaplastic large cell lymphoma (ALCL), specifically anaplastic lymphoma kinase (ALK)-positive (ALK+ ALCL), is an aggressive CD30-positive T-cell lymphoma characterized by ALK gene translocations and protein expression.
- While no specific risk factors are identified, ALK+ ALCL presents with distinct morphologic features, including hallmark cells, and typically affects younger males, often at advanced stages.
Purpose of the Study:
- To summarize the key characteristics, prognostic factors, and current treatment strategies for ALK+ ALCL.
- To highlight the efficacy of standard chemotherapy and explore the role of emerging therapies.
Main Methods:
- Review of existing literature on ALK+ ALCL, including morphologic patterns, clinical presentation, and prognostic indicators.
- Analysis of treatment outcomes from standard first-line polychemotherapy regimens and consolidative high-dose chemotherapy with autologous stem cell transplantation (HDC/ASCT).
Main Results:
- ALK+ ALCL shows a better prognosis than other T-cell lymphomas, with survival predicted by International Prognostic Index (IPI) and PIT scores.
- Standard doxorubicin-containing polychemotherapy achieves high response rates (∼90%) and 5-year survival rates of approximately 70%.
- HDC/ASCT is effective for relapsed/refractory cases but its superiority over standard chemotherapy is unproven; novel therapies targeting CD30 and ALK are emerging.
Conclusions:
- ALK+ ALCL is a distinct entity with a relatively favorable prognosis compared to other T-cell lymphomas, manageable with current chemotherapy regimens.
- While standard chemotherapy is effective, ongoing research into novel targeted therapies offers hope for improved outcomes in relapsed or refractory ALK+ ALCL.