Duong Van Huyen J P, Molina T, Delmer A, Audouin J, Le Tourneau A, Zittoun R, Bernadou A, Diebold J
Department of Pathology, H tel Dieu, AP-HP, Paris, France.
Am J Surg Pathol. 2000 Dec;24(12):1581-92. doi: 10.1097/00000478-200012000-00001.
We report a series of 31 cases of splenic marginal zone lymphomas with an enlarged spleen and a multimicronodular macroscopic pattern. Two groups, A and B, were distinguished based on the presence (A) or absence (B) of a lymphoplasmacytic component with monoclonal immunoglobulin expression in the cytoplasm. There were no differences between the groups as far as age, sex, spleen weight, and progression. The only difference was the presence in group A of a monoclonal serum component and autoimmune disorders, particularly autoimmune hemolytic anemia. In most cases in which a liver and/or bone marrow biopsy was performed, lymphomatous infiltration was detected. Seven cases had a seric monoclonal IgM of 5 g/L or more and liver or bone marrow infiltration, corresponding to the definition of Waldenstrom's macroglobulinemia. Lymphoma cells had a monocytoid, centrocytoid and, in group A, lymphoplasmacytic morphology. The lymphomatous cells were positive for CD20, CD45 RA, and bcl-2. They expressed IgD in 9 cases, partially in 6, and were negative for IgD in 9 of the 24 cases studied. Progression seems to be slow, with a long survival. Three patients presented with transformation into a large B-cell lymphoma, which was responsible for death in two patients.
我们报告了31例脾边缘区淋巴瘤病例，这些病例脾脏肿大，具有多微结节的大体形态。根据细胞质中是否存在具有单克隆免疫球蛋白表达的淋巴浆细胞成分，将病例分为A、B两组。两组在年龄、性别、脾脏重量和病情进展方面无差异。唯一的差异是A组存在单克隆血清成分和自身免疫性疾病，尤其是自身免疫性溶血性贫血。在大多数进行肝脏和/或骨髓活检的病例中，均检测到淋巴瘤浸润。7例患者血清单克隆IgM≥5 g/L且伴有肝脏或骨髓浸润，符合华氏巨球蛋白血症的定义。淋巴瘤细胞具有单核样、中心细胞样形态，在A组中还有淋巴浆细胞形态。淋巴瘤细胞CD20、CD45 RA和bcl-2呈阳性。在24例研究病例中，9例IgD表达阳性，6例部分表达，9例IgD阴性。病情进展似乎缓慢，生存期较长。3例患者转化为大B细胞淋巴瘤，其中2例因此死亡。
