ALK-positive anaplastic large cell lymphoma: Statistics and survival trends.

Abstract

7078 Background: Anaplastic lymphoma kinase positive anaplastic large cell lymphoma (ALK+ ALCL) is a rare subtype of peripheral T-cell lymphoma, characterized by CD 30+ large pleomorphic lymphoid cells with horseshoe nuclei and abundant cytoplasm. It frequently involves t(2;5) combining ALK with nucleophosmin (NPM1) gene. ALK+ ALCL is chemotherapy responsive, and CHOP, CHOEP (CHOP+etoposide) or BV-CHP (brentuximab instead of vincristine) are the popular drug combinations used. Methods: We extracted ALK+ ALCL cases using the ICD Code 9714/3, from Surveillance, Epidemiology and End Result (SEER) database Research Plus Data, 17 Registries, Nov 2023 Sub (2000-2021). The analysis was stratified based on age, sex, race, primary site labelled, laterality, stage, median household income inflation adjusted to 2022, and treatment options utilized. Survival curves were compared using the Log-Rank test (GraphPad Prism). Results: Total 3916 cases of ALK+ ALCL were identified, with median age at diagnosis of 53.5 years. Of the cases, 61% were males. Racial distribution was noted as: Caucasians 63.2%, Hispanics 16.1%, Blacks 12.4%, Asian/Pacific Islanders 6.7%, American Indians/Alaskan and unknown race were <1%, each. Overall median of survival (MoS) was 118 months, with 1-year OS of 0.696 (CI 95%, 0.68-0.71), 3-year OS of 0.61 (CI 95%, 0.596-0.63), and 5-year OS of 0.57 (CI 95%, 0.56-0.59). MoS were significant for Age: 0-30 yrs (undefined), 31-60 (233), 60+ yrs (17) (p <0.0001); Gender: males (94) and females (153) (p 0.0011); Race: White (119), Black (49), Hispanics (118), Asian/Pacific Islanders (180), Alaskan/Native Americans (87), and unknown race (undefined) (p <0.0001); Laterality: right (162), left (173), bilateral (144), and unknown side (66) (p <0.0001). Survival based on stage was undefined for loco-regional disease, 67 for distant and 65 months for unknown stage (p <0.0001). Anatomically, analysis revealed higher MoS with connective tissue (179), head/face/neck (72), and lymphoid origin (119); while lower survival with GI (21), thorax (13), and unknown site (7) (p <0.0001). MoS improved with increasing income, <70,000$ (73), 70K-100K (129), and >100K (135) (p <0.0001). Treatment based analysis showed: surgery (151) vs no surgery (100) (p <0.0001), chemotherapy (182) vs no chemo (27) (p <0.0001), radiotherapy (XRT) (173) vs no XRT (108) (p <0.0001). Undefined MoS were likely observed due to insignificant numbers of death in those age categories to calculate 50% survival probability. Conclusions: ALK+ ALCL is a rare malignancy that favors male gender, and Caucasian race. Our analysis revealed superior survival outcomes associated with younger age, female sex, Asian/Pacific Islander origin, connective tissue involvement, unilateral and loco-regional disease, and treatment involving either surgery or non-surgical options, specifically chemotherapy. This is the first study to our knowledge to establish association of ALK+ ALCL to income, showing higher survival with increasing income bracket.