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Breaking diagnostic and therapeutic barriers in intravascular large B-cell lymphoma: A 13-year real-world study from China
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Breaking diagnostic and therapeutic barriers in intravascular large B-cell lymphoma: A 13-year real-world study from China
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Journal Article
Breaking diagnostic and therapeutic barriers in intravascular large B-cell lymphoma: A 13-year real-world study from China
2026
Overview
Background
Intravascular large B-cell lymphoma (IVLBCL) is an ultra-orphan disease (incidence 0.095/million/year) with dismal prognosis due to delayed diagnosis and limited therapeutic options. We aimed to evaluate a novel diagnostic algorithm and the efficacy of zanubrutinib-containing therapy in a real-world Chinese cohort.
Methods
This single-center retrospective study enrolled 54 IVLBCL patients (2010–2022). Diagnostic approaches evolved from PET/CT-guided biopsy to a combination of serum interleukin-10 (IL-10, cut-off 95.65 pg/mL), random skin biopsy (RSB), and circulating tumor DNA (ctDNA) profiling. Treatment regimens shifted from R-CHOP to methotrexate-based therapy (MTX), then to zanubrutinib plus R-CHOP (ZR-CHOP).
Results
The cohort exhibited distinct features: 35.2% Asian-variant IVLBCL, 50% CNS involvement, and 22.9% PET/CT negativity. IL-10 combined with RSB enabled the diagnosis of 11 PET/CT-negative patients who would have otherwise remained undiagnosed. ctDNA revealed
MYD88 L265P
(11/17, 65%) and
CD79B
(7/17, 41%) variants. ZR-CHOP (
n
= 22) achieved significantly superior 2-year progression-free survival (PFS) compared to R-CHOP alone (
n
= 6) (90% vs. 30%; hazard ratio [HR] 0.031,
P
< 0.0001) and demonstrated 100% central nervous system (CNS) relapse-free survival. The efficacy of ZR-CHOP was comparable to that of methotrexate (MTX)-based therapy (
n
= 20; 2-year PFS 85%), despite a shorter median follow-up (20.1 vs. 38.0 months).
Conclusions
In this largest Asian IVLBCL cohort to date, IL-10 + RSB + ctDNA significantly improved diagnostic accuracy. Zanubrutinib demonstrated promising efficacy, particularly in CNS involvement, offering a pragmatic solution for this orphan disease.
Publisher
BioMed Central,Springer Nature B.V,BMC
Subject
/ Aged
/ Antineoplastic Combined Chemotherapy Protocols - therapeutic use
/ Biopsy
/ China
/ Circulating Tumor DNA - blood
/ Cyclophosphamide - therapeutic use
/ Doxorubicin - therapeutic use
/ Female
/ Humans
/ Hypoxia
/ Intravascular large B-cell lymphoma
/ Lymphoma
/ Lymphoma, Large B-Cell, Diffuse - diagnosis
/ Lymphoma, Large B-Cell, Diffuse - drug therapy
/ Male
/ Medicine
/ Methotrexate - therapeutic use
/ Patients
/ Positron Emission Tomography Computed Tomography
/ Prednisone - therapeutic use
