Review Article The biology and management of systemic anaplastic large cell lymphoma

Systemic anaplastic large cell lymphoma (ALCL) is an aggressive CD301 non-Hodgkin lymphoma. Anaplastic lymphoma kinase–positive (ALK1) ALCL is associ-atedwith theNPM-ALK t(2;5) translocation, which is highly correlated with the identifi-cation of the ALK protein by immunohisto-chemistry. ALK1 ALCL typically occurs in younger patients and has a more favorable prognosis with 5-year survival rates of 70% to 90 % in comparison with 40 % to 60 % for ALK-negative (ALK2) ALCL. Studies sup-port young age as a strong component of the favorable prognosis of ALK1 ALCL. Until recently, no recurrent translocation