Audouin Josée, Le Tourneau Agnès, Molina Thierry, Camilleri-Broët Sophie, Adida Colette, Comperat Eva, Benattar Laurence, Delmer Alain, Devidas Alain, Rio Bernard, Diebold Jacques
Service central Jacques-Delarue d'Anatomie et de Cytologie Pathologiques, Hôtel Dieu, Paris, France.
Br J Haematol. 2003 Aug;122(3):404-12. doi: 10.1046/j.1365-2141.2003.04449.x.
We studied 86 bone marrow biopsies (BMB) from 58 patients presenting with primary splenic marginal zone lymphoma (PSMZL). In 42 patients, a splenectomy was performed which enabled a histopathological diagnosis. In these patients, 44 biopsies were carried out before, and 25 after, splenectomy. In 16 recently observed patients, 17 BMB led to PSMZL diagnosis, and these patients were treated without splenectomy. Seven different patterns of infiltrates were recognized: intravascular, interstitial, nodular, massive, plasmacytic mimicking myeloma and transformation into large B-cell lymphoma (DLBCL). The association of an intravascular infiltrate and nodules with a germinal centre and/or a marginal zone favoured a diagnosis of MZL. Immunohistochemistry demonstrated the expression of B cell-associated antigens and, in 40% of the patients, a monotypic lymphoplasmacytic cell component. These patients often presented a serum M component and autoimmune disorders. In the past, such cases have been diagnosed as lymphoplasmacytic lymphoma. BM involvement was present in all patients. Successive biopsies showed progression and, after chemotherapy, a slight decrease in infiltrates. Transformation into DLBCL occurred in 11 of 34 patients. The patterns described are not specific for PSMZL and occur also in primary nodal MZL and, more rarely, in MALT-type lymphoma.
我们研究了58例原发性脾边缘区淋巴瘤（PSMZL）患者的86份骨髓活检（BMB）样本。42例患者接受了脾切除术，从而得以进行组织病理学诊断。在这些患者中，44份活检在脾切除术前进行，25份在脾切除术后进行。在16例近期观察的患者中，17份BMB样本确诊为PSMZL，这些患者未接受脾切除术即接受了治疗。识别出七种不同的浸润模式：血管内、间质、结节状、弥漫性、模仿骨髓瘤的浆细胞样以及转化为大B细胞淋巴瘤（DLBCL）。血管内浸润和结节与生发中心和/或边缘区的关联有利于MZL的诊断。免疫组织化学显示B细胞相关抗原的表达，40%的患者存在单型淋巴浆细胞成分。这些患者常出现血清M成分和自身免疫性疾病。过去，此类病例被诊断为淋巴浆细胞淋巴瘤。所有患者均有骨髓受累。连续活检显示病情进展，化疗后浸润略有减少。34例患者中有11例转化为DLBCL。所描述的模式并非PSMZL所特有，也见于原发性淋巴结MZL，在MALT型淋巴瘤中则更为少见。
