Authors
Shuchao Qin, Jingxin Zhou, Yi Miao, Yi Xia, Luomengjia Dai, Ziyuan Zhou, Tonglu Qiu, Ming Liu, Lei Fan, Jianyong Li, Huayuan Zhu.
Objective
To analyze the clinical characteristics and treatment of chronic lymphocytic leukemia (CLL) patients with autoimmune cytopenia.
Methods
A retrospective analysis was conducted on 39 CLL patients with autoimmune cytopenia treated at The First Affiliated Hospital of Nanjing Medical University between July 2011 and September 2024. Clinical features and treatment strategies were evaluated.
Results
Among the 39 CLL patients, 26 (67%) had autoimmune hemolytic anemia (AIHA), 12 (31%) had immune thrombocytopenia (ITP), and 1 (3%) had Evans syndrome. The cohort included 23 males (59%) and 16 females (41%), with a median age of 61 years (range: 43–87) at CLL diagnosis. According to the CLL-International Prognostic Index (CLL-IPI), 3 patients (8%) were low-risk, 11 (28%) intermediate-risk, 15 (38%) high-risk, and 10 (26%) very high-risk. Monoclonal proteins were detected by immunofixation electrophoresis in 7 patients (18%). Six patients (15%) had concurrent autoimmune diseases (e.g., systemic lupus erythematosus, dermatomyositis, psoriasis). Twenty-five patients (64%) were with mutated IGHV and 14 (36%) were with unmutated IGHV. Genetic abnormalities included TP53 aberrations in 9 (23%), NOTCH1 mutations in 4 (10%), and SF3B1 mutations in 5 (13%). Regarding disease progression: 22 patients (56%) were diagnosed with CLL and cytopenia simultaneously; 3 (8%) were diagnosed with ITP first, with CLL diagnosed 1, 4, and 10 years later; 13 (33%) were diagnosed with CLL first—8 developed cytopenia during watch-and-wait (median time from CLL diagnosis to ITP-directed therapy: 71.5 months; 7 AIHA, 1 ITP), while 4 developed cytopenia post-CLL treatment (2 AIHA, 2 ITP). With a median follow-up of 49 months (range: 8–166), 28 patients (72%) initiated CLL-directed therapy.
Conclusion
CLL patients with autoimmune cytopenia exhibit significant clinical heterogeneity and variable sequences of diagnosis. Suboptimal response to initial cytopenia-directed therapy in some patients necessitates timely initiation of CLL-targeted treatment for disease control.
Keywords : Autoimmune cytopenia; Autoimmune hemolytic anemia; Immune thrombocytopenia; Treatment
Please indicate how this research was funded. :
Please indicate the name of the funding organization.: