Abstract: Objective To analyze the clinical features, response to therapy and prognosis of intravascular large B-cell lymphoma (IVLBCL). Methods The clinical data of 17 cases with IVLBCL were retrospectively reviewed, and survival analysis was conducted. Results The study involved 10 males and 7 females of IVLBCL with a mean age of 53 years old. The most common symptom of the disease was recurrent fever (76.5%). The lymphoma was mainly observed in bone marrow (64.7%) and was clinically determined as stage ⅣB (70.6%). Many of the patients were also diagnosed with the hemophagocytic syndrome (29.4%). R-CHOP (rituximab, cyclophosphamide, epirubicin, vindesine, prednisone) or CHOP regimen chemotherapy significantly improved the survival of the patients (P=0.000 2). Unfortunately, those patients with bone marrow involvement were prone to relapse after treatment. Conclusion IVLBCL is highly invasive and associated with poor prognosis. R-CHOP chemotherapy can significantly improve the prognosis.