Zhang Xiaohui, Sun Manhua, Zhang Ling, Shao Haipeng
Hematopathology and Laboratory Medicine, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.
Int J Clin Exp Pathol. 2013 Apr 15;6(5):985-9. Print 2013.
De novo CD5-positive diffuse large B-cell lymphoma (CD5(+) DLBCL) accounts for approximately 10% of DLBCL, and is usually associated with aggressive clinical course. We report a case of CD5(+) DLBCL with primary involvement of the spleen and liver, and no distinct mass lesions or lymphadenopathy. The patient had stage IV disease with bone marrow involvement by lymphoma. The lymphoma cells showed characteristic portal and intrasinusoidal pattern of infiltrate in the liver. The literature was reviewed and the clinicopathologic features of 7 similar reported cases were summarized. All cases share the common features of hepatosplenomegaly without mass lesions, exclusive red pulp infiltrate with a diffuse and cordal pattern in the spleen, portal and intrasinusoidal pattern of infiltrate in the liver, and stage IV disease with poor response to conventional chemotherapy. This may represent a distinct subgroup of CD5(+) DLBCL and the diagnosis is important for prompt clinical treatment.
原发性CD5阳性弥漫性大B细胞淋巴瘤（CD5(+) DLBCL）约占DLBCL的10%，通常与侵袭性临床病程相关。我们报告1例CD5(+) DLBCL，主要累及脾脏和肝脏，无明显肿块或淋巴结病。该患者为IV期疾病，淋巴瘤累及骨髓。淋巴瘤细胞在肝脏呈现特征性的汇管区和窦内浸润模式。回顾了相关文献并总结了7例类似报道病例的临床病理特征。所有病例均具有肝脾肿大但无肿块、脾脏仅红髓浸润呈弥漫性和条索状、肝脏呈汇管区和窦内浸润模式、IV期疾病且对传统化疗反应不佳的共同特征。这可能代表CD5(+) DLBCL的一个独特亚组，其诊断对及时的临床治疗很重要。
