Abstract: Objective To investigate the differences in clinical manifestations, diagnosis, treatment and prognosis among different subtypes of intravascular large B-cell lymphoma (IVLBCL) in China. Methods A total of 185 cases of IVLBCL which were reported in the literature from 1994 to 2021 in China were systematically retrieved and retrospectively analyzed. Results A total of 178 cases of 185 IVLBCL patients were diagnosed by biopsy, of which 61.8% (81/131) had two or more organs involved. Initial symptoms and ancillary examination results were highly diversified. Among all these cases, 35 were central nervous system (CNS) involvement, 29 hemophagocytic syndrome (HPS) involvement, 78 other involvement, and 43 unknown. Compared with the HPS involvement, the CNS involement had lower incidence rates of fever (25.7% vs 86.2%, P<0.001), anemia/thrombocytopenia (50.0% vs 100.0%, P=0.004), bone marrow invasion (21.4% vs 93.1%, P<0.001), and the rate of receiving chemotherapy (40.0% vs 84.0%, P=0.001). The prognosis of the CNS involvement was worse than that of the HPS involvement (2.0 months vs not reached, P<0.001) and others (2.0 months vs 23.0 months, P<0.001). The 1-year survival rate of the chemotherapy group was higher than that of the non-chemotherapy group (53.4% vs 2.7%, P<0.001). Conclusions The clinical manifestation of IVLBCL in China is highly diversified. The prognosis of the CNS involvement is worse than that of the HPS involvement, and chemotherapy can significantly improve the prognosis.

Key words: Intravascular lymphoma, Large B-cell, Clinical variant, Central nervous system lymphoma