CLINICAL PRESENTATION, OUTCOME, AND PROGNOSTIC FACTORS IN PATIENTS WITH INTRAVASCULAR LARGE B‐CELL LYMPHOMA: THE ASIA EXPERIENCE FROM A SINGLE‐CENTER RETROSPECTIVE STUDY

Introduction: Intravascular large B-cell lymphoma (IVLBCL) is a rare, clinically aggressive for which there no available standard treatment. IVLBCL characterized by selective growth in the lumina of small vessels systemic organs usually presents with many nonspecific signs and symptoms such as fever unknown origin involvement central nervous system skin. Methods: This retrospective, single-center study 59 patients. Only HIV-negative, immunocompetent patients untreated were included. The demographics, diagnostic details, therapeutic management, outcome analyzed. Results: identified PUMCH from December 2010 to February 2023. median age at diagnosis was 58 (range 32–77). 30 (51.8%) male. 43 (72.9%) had an IPI score >3 47 (79.7%) performance status ≥2. All belonged Ann Arbor stage IV 52(88.1%) B symptoms. most frequent extra-nodal locations skin (n = 30; 50.8%), lung 25; 42.4%), 24; 40.7%), bone marrow 20; 33.9%). Fever, hypoxemia, edema common presenting Diagnosis made random biopsy (25), other lesions (15), (11) TBLB (8). Plasma cell-free DNA has been detected 17 these frequently mutations PIM-1 (76%), MYD88 (59%), CDKN2B (59%) TNFAIP3 (53%). 40 who have serum IL-10 concentration elevated (≥5 pg/mL, range 11.5–1000 pg/mL). 23 received zanubrutinib plus R-CHOP therapy, 22 regimen anthracycline-based chemotherapy containing rituximab, 5 without Rituximab. 18 all also intrathecal or methotrexate prevent CNS relapse. After follow-up 22.8 months, estimated progression-free survival overall entire cohort 119.8 122.9 months. use zanubrutuinib not associated better PFS OS. research funded by: National High Level Hospital Clinical Research Funding, 2022-PUMCH-A-192. CAMS Innovation Fund Medical Sciences (CIFMS), 2021-I2M-C&T-B-005 Keywords: non-Hodgkin lymphoma, No conflicts interests pertinent abstract.