Objective To investigate the clinicopathological features of intravascular large B-cell lymphoma(IVLBCL).Methods A retrospective analysis was performed for the clinical data of four patients with IVLBCL,and a literature review was also conducted to analyze the clinicopathological features of this disease.Results All four patients had the clinical manifestation of pyrexia,among whom two had pulmonary infiltration,one had skin changes,and one had central nervous system infiltration.Laboratory tests showed abnormal routine blood test results in all four patients(three cases of anemia,two cases of thrombocyto-penia,and one case of leucopenia),an increase in lactate dehydrogenase in four patients,hypoproteinemia in three patients,and an increase in interleukin-10 in three patients.Radiological examination showed negative PET-CT results in two patients.All four pa-tients had stage Ⅳ IVLBCL according to the Ann Arbor staging system.A confirmed diagnosis was made based on biopsy,and among the four patients,two underwent bone marrow biopsy,one underwent uterus biopsy,and one underwent blind skin biopsy.All four patients had the clinical manifestation of lymphoma cells confined to the lumen of small blood vessels,which were mature B-cells,and the Hans model was a non-germinal center.CD5 expression was observed in three patients.Conclusion IVLBCL is a rare disease with diverse clinical features,and pyrexia is the main clinical feature,with or without involvement of lungs,skin,and the nervous system.PET-CT often has negative results,thereby leading to misdiagnosis and missed diagnosis,and a confirmed diagnosis requires bone marrow biopsy,skin biopsy,or biopsy of the involved organs.
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