Molecular Profiling of Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphoma (ALCL) is a non-Hodgkin’s lymphoma (NHL); NHL includes many cancers of white blood cells. Approximately 750-800 children are diagnosed with NHL each year in the U.S. (SEER 1999), and about 13% of these are diagnosed with ALCL (Drexler et al. 2000). The five-year survival rate for children diagnosed with NHL is 72% (SEER 1999). In all age groups, 72,580 cases of NHL were estimated for 2016, and 20,150 deaths (ACS 2016). ALCL makes up approximately 2% of adult NHL (Drexler et al. 2000).

ALCL was identified in the 1980s, and it was first included in the WHO classification system in 2001. Characteristics include hallmark cells and CD30 positivity (Drexler et al. 2000). Hallmark cells have eccentric, kidney-shaped, or horseshoe-shaped nuclei (Ferreri et al. 2012). CD30 is a receptor protein expressed on the surface of activated T and B cells. Treatment of childhood ALCL depends on stage: low-stage ALCL is treated with several cycles of chemotherapy (NCI 2012), while high-stage ALCL is treated with additional cycles of chemotherapy (NCI 2012). Bone marrow transplantation is used for recurrent ALCL.

ALCL is further divided into ALK-positive and ALK-negative ALCL (Falini and Martelli 2009). The predominant genetic alteration observed in ALCL is the NPM1-ALK fusion seen in 31% of adult and 83% of pediatric ALCL patients (Drexler et al. 2000). Development of targeted therapeutics for ALK-positive ALCL has focused on ALK (Ferreri et al. 2012). CD30 antibodies have been explored as a potential treatment in ALK-positive and ALK-negative ALCL (Merkel et al. 2011).

Contributors: Valerie Brown, M.D., Ph.D., Scott C. Borinstein, M.D., Ph.D., Debra Friedman, M.D.

Suggested Citation: Brown, V., S. Borinstein, D. Friedman. 2016. Molecular Profiling of Anaplastic Large Cell Lymphoma. My Cancer Genome https://www.padiracinnovation.org/content/disease/anaplastic-large-cell-lymphoma/ (Updated January 26).

Last Updated: January 26, 2016

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