Primary systemic anaplastic lymphoma kinase negative anaplastic large cell lymphoma: clinical analyses of 32 cases

Abstract

Objective To investigate the clinical manifestations, treatment outcomes and prognosis factors of the patients with primary systemic anaplastic lymphoma kinase negative anaplastic large cell lymphoma (ALK- ALCL). Methods The clinical data of 32 patients histologically confirmed with ALK- ALCL from January 2011 to December 2017 in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed. Kaplan-Meier method, Log-rank test and Cox proportional hazards model were used for the survival analysis. Results There were 19 males and 13 females in 32 patients, and the median age of onset was 50 years old (14-78 years old). The proportion of the patients with B symptom, extranodal involved, stage Ⅲ-Ⅳ, International Prognostic Index (IPI) sore 2-3 were 34.4% (11 cases), 53.1% (17 cases), 59.4% (19 cases) and 50.0% (16 cases) respectively. Chemotherapy alone and chemotherapy plus radiotherapy were the main methods, and a few patients received surgery alone, surgery plus chemotherapy, chemotherapy plus autologous stem cell transplantation or palliative care. The efficacy of 28 cases could be evaluated. The objective effective rate was 85.7% (24/28), the complete remission (CR) rate was 42.9% (12/28), and the partial remission (PR) rate was 42.9% (12/28). The median progression-free survival (PFS) time was 28 months (95% CI 13-42 months) and the median overall survival (OS) time was 43 months (95% CI 24-61 months). The anticipated 5-year OS rate was 36.6%. Single factor analysis results revealed that patients who was female (χ 2= 7.654, P= 0.006), with normal serum lactic dehydrogenase (LDH) level (χ 2= 7.575, P= 0.006), normal serumβ2 microglobulin level (χ 2= 4.770, P= 0.029) and without B symptom (χ 2= 9.418, P= 0.002), IPI score 0-1 (χ 2= 4.119, P= 0.042) had a better prognosis. Multivariate survival analysis indicated that symptoms B (HR= 12.460, 95% CI 2.645-58.708, P= 0.001) and IPI score (HR=7.521, 95% CI 1.624-34.841, P= 0.010) were the independent factors for prognosis. Conclusion The prognosis of the patients with primary systemic ALK- ALCL is poor, especially for the worse prognosis of the patients with symptoms B and IPI sore 2-3. Key words: Lymphoma, large-cell, anaplastic; Anaplastic lymphoma kinase; Prognosis