Li Shiyong, Mann Karen P, Holden Jeannine T
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA 30322, USA.
Int J Surg Pathol. 2004 Jan;12(1):31-7. doi: 10.1177/106689690401200105.
We review the clinical, pathologic, and molecular genetic features of 3 splenic T-cell-rich B-cell lymphomas and discuss their differential diagnosis. All patients presented with symptomatic splenomegaly and underwent diagnostic/therapeutic splenectomy. Microscopically, the spleen in all cases showed a micronodular proliferation of lymphoid cells. A proportion of the nodules demonstrated central hyalinization or sclerosis. There was also an exuberant extramedullary hematopoiesis. On immunohistochemical stain, the nodules consisted predominantly of small T cells with scattered large atypical B cells. The clonal nature of the atypical B cells was confirmed by polymerase chain reaction assays for immunoglobulin heavy-chain gene rearrangement. In the H&E sections, the differential diagnoses included Hodgkin's lymphoma, follicular lymphoma, peripheral T-cell lymphoma, and nonneoplastic granulomatous process. The presence of exuberant extramedullary hematopoiesis also raised the possibility of a chronic myeloproliferative disorder. The combined morphologic, immunohistochemical, and molecular genetic data are essential for a correct diagnosis of splenic T-cell-rich B-cell lymphoma.
我们回顾了3例脾T细胞丰富的B细胞淋巴瘤的临床、病理及分子遗传学特征，并讨论了它们的鉴别诊断。所有患者均表现为有症状的脾肿大，并接受了诊断性/治疗性脾切除术。显微镜下，所有病例的脾脏均显示淋巴细胞呈微结节状增生。部分结节呈现中央透明变性或硬化。同时还存在旺盛的髓外造血。免疫组化染色显示，结节主要由小T细胞和散在的大的非典型B细胞组成。通过免疫球蛋白重链基因重排的聚合酶链反应检测证实了非典型B细胞的克隆性质。在苏木精-伊红（H&E）切片中，鉴别诊断包括霍奇金淋巴瘤、滤泡性淋巴瘤、外周T细胞淋巴瘤和非肿瘤性肉芽肿性病变。旺盛的髓外造血的存在也增加了慢性骨髓增殖性疾病的可能性。综合形态学、免疫组化和分子遗传学数据对于正确诊断脾T细胞丰富的B细胞淋巴瘤至关重要。
