Intravascular large B-cell lymphoma: clinical and pathological analyses of three cases

ZENG Ying; MA Qiang; ZHAO Lian-hua; DU Juan; MA Yu; ZHONG Peng; LIN Li; FU Ping; XIAO Hua-liang

doi:10.3969/j.issn.1007-8096.2017.08.004

Abstract：Objective To investigate clinicopathologic features and differential diagnosis of intravascular large B-cell lymphoma (IVLBCL).Methods The clinical and radiological data were collected in three cases of IVLBCL,their pathological and immunohistochemical features were analyzed,with discussion of the pathological diagnosis and differential diagnosis based on related literature.Results The age of the patients at diagnosis ranged from 51 to 78 years;two cases were male and one case was female.The two lesions were located in the adrenal glands,and one lesion was located in the right parietal lobe of the brain.Clinical manifestation of the patients in the adrenal gland was back pain or discomfort;the patient in the fight parietal lobe showed dizziness and headache.Microscopic observation showed morphological findings were similar in the 3 cases:tumor cells located in small vascular lumina,and large blood vessels could also be involved.Tumor cells were large,nuclear vacuoles,and prominent nucleoli.High mitosis and fibrin thrombus were seen.Immunohistochemistry showed that the tumor cells were positive for CD20,CD79a,PAX-5,Bcl-6,MUM-1 and bcl-2;but negative for CK,CD3,CD5,CD30,CD10,EBER,Syn,SALL4,OCT3/4,CD117 and PLAP;C-Myc positive nucleus ratio was 10％～20％;Ki-67 proliferation index was 30％,40％,and 80％,respectively.Conclusion IVLBCL is a rare variant of diffuse large B cell lymphoma.Differential diagnosis includes other small cell malignant tumors.The diagnosis should be combined with histopathological changes and immunohistochemical phenotype.The tumor is highly malignant and aggressive,and not sensitive to chemotherapy;most patients die in a short time after diagnosis,and so prognosis is poor.Clinical manifestations are nonspecific and early diagnosis is difficult.Clinicians should arouse vigilance,try their best to diagnose early and chemotherapy can prolong the patient's survival time.