血管内大B细胞淋巴瘤4例临床病理特征分析

Clinicopathological features of intravascular large B-cell lymphoma:An analysis of four cases

目的探讨血管内大B细胞淋巴瘤(IVLBCL)的临床病理特征。方法回顾分析4例IVLBCL病人的临床资料,并结合文献复习探讨该病的临床病理特征。结果4例病人的临床表现均为发热,其中有肺部浸润2例,皮肤改变1例,中枢浸润1例。实验室检查显示4例病人血常规均异常(贫血3例,血小板减少2例,白细胞减少1例),4例乳酸脱氢酶升高,3例低蛋白血症,3例白细胞介素-10升高。影像学检查2例PET-CT阴性。4例病人的Ann Arbor分期均为Ⅳ期。通过病理活检确诊,其中骨髓活检2例,子宫活检1例,皮肤盲检1例。4例病人病理均表现为淋巴瘤细胞局限于小血管腔内生长,均为成熟B细胞,Hans模型为非生发中心,3例伴有CD5表达。结论IVLBCL罕见,临床特征多变,以发热为主要特征,伴或不伴肺部、皮肤和神经系统受累。PET-CT检查多为阴性,易误诊漏诊,确诊需要骨髓活检、皮肤活检或受累器官活检。

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 thrombocytopenia,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 patients had stageⅣIVLBCL 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.