Clinical features and prognostic outcomes of systemic anaplastic large cell lymphoma in a Chinese multicenter study

Objective The prevalence of systemic anaplastic large cell lymphoma (sALCL) is low and remains under-investigated. This study aimed to investigate the clinical features, treatment outcomes, and prognostic factors of sALCL. Methods A retrospective analysis of 143 sALCL patients treated at 14 centers from 2010 to 2022 was conducted. Results The median age was 44 years, with a male predominance. Most patients were diagnosed at an advanced stage, and extranodal involvement, particularly in the bone, was observed in 51.0% of cases. Most patients exhibited elevated LDH, β2-MG, CD30 positivity, and high Ki-67 expression. ALK-positivity was found in 55.9% of patients, who were significantly younger than ALK-negative patients ( p  < 0.001). First-line chemotherapy achieved a complete response (CR) rate of 66.7% and an overall response rate (ORR) of 78.5%. CHOPE was more effective than CHOP. Failure to achieve complete response, age ≥ 60 years, bone marrow involvement, and elevated β2-MG were identified as independent prognostic factors for survival. Conclusion This study highlights the unique distinct clinical and molecular features of sALCL in the real world of China and suggests that ALK expression and treatment regimen significantly influence outcomes, providing new data for clinical practice.