Clinicpathological and molecular of intravascular large B-cell lymphoma
SUI Yuming
CHEN Huang
NIU Yiru
GUO Jia
ZHAO Lingyu
ZHONG Dingrong
Abstract：Objective To explore the clinical and pathological features of intravascular large B-cell lymphoma(IVLBCL)and the status of MYD88 and CD79B gene mutations.Methods The clinical and pathological data of 10 patients with IVLBCL diagnosed at China-Japan Friendship Hospital from 2018 to 2021 were collected and analyzed by HE staining,immunohistochemistry and next-generation sequencing.Results The median age of the patients was 58.7 years,with a majority being female(7 cases).The involved sites included the lung,skin,bone marrow and liver.Pathological examination showed that tumor cells mainly aggregated in small blood vessels.Immunohistochemistry revealed diffuse positivity for CD20 and CD79a,and a high Ki-67 proliferation index.Gene testing found MYD88 mutations in 20%of cases and CD79B mutations in 60%,with one case having both mutations.Five patients were Lugano stage Ⅳ,and one died during hospitalization.Three patients received R-CHOP plus intrathecal methotrexate,one received R-CHOP,one received R-miniCHOP,and one received rituximab once.During follow-up,four patients survived and two died.Conclusion IVLBCL is an aggressive B-cell lymphoma with non-specific and insidious clinical manifestations,which is prone to misdiagnosis.The pathological morphology is diverse.MYD88 and CD79B mutations may be related to its pathogenesis.Treatment regimen BTK inhibitors deserve further exploration.Early multi-site biopsy(such as random skin biopsy)is crucial for improving the diagnos rate.Imaging studies can provide for biopsy sites.Advances in molecular pathology and treatment regimens improve survival rates.
Keywords：Intravascular large B-cell lymphomaClinical and pathological featuresImmunohistochemistryMYD88CD79B
Publication Date：2025-12-28
Online Publishing Date：2026-01-28(First online date of this platform, not the publication date of the document)
Pages：6( 1611-1616 )