Carulli Giovanni, Marini Alessandra, Ciancia Eugenio M, Bruno Joseph, Vignati Silvana, Lambelet Paola, Cannizzo Elisa, Ottaviano Virginia, Galimberti Sara, Caracciolo Francesco, Ferreri Maria I, Ciabatti Elena, Petrini Mario
Department of Oncology, Transplants and New Technologies in Medicine, Division of Hematology and Section of Flow Cytometry, University of Pisa, Pisa, Italy.
J Med Case Rep. 2011 Sep 23;5:476. doi: 10.1186/1752-1947-5-476.
Discordant lymphomas are rare entities characterized by the simultaneous presence of two distinct types of lymphomas in different anatomic sites. We describe a very rare case of simultaneous occurrence of splenic mantle cell lymphoma and marginal zone lymphoma involving the bone marrow and peripheral blood.
We report the case of a 60-year-old asymptomatic Caucasian woman in whom discordant lymphomas were discovered when a slight lymphocytosis and a conspicuous splenomegaly were observed. The different morphological, immunophenotypical and immunohistochemical features found in the different pathologic samples obtained from peripheral blood, bone marrow and spleen sections made it possible to differentiate two types of non-Hodgkin B-cell lymphomas: a mantle cell lymphoma infiltrating the spleen and a marginal zone lymphoma involving both the bone marrow and peripheral blood. Since a similar IgH gene rearrangement was found both in the bone marrow and in the spleen, the hypothesis of a common origin, followed by a different clonal selection of the neoplastic lymphocytes may be taken into consideration.
Our case emphasizes the usefulness of investigating simultaneous specimens from different anatomic sites from the same patient and the relevant diagnostic role of splenectomy.
异质性淋巴瘤是罕见的实体瘤,其特征是在不同解剖部位同时存在两种不同类型的淋巴瘤。我们描述了一例非常罕见的同时发生脾套细胞淋巴瘤和累及骨髓及外周血的边缘区淋巴瘤的病例。
我们报告了一例60岁无症状的白种女性病例,在观察到轻度淋巴细胞增多和明显脾肿大时发现了异质性淋巴瘤。从外周血、骨髓和脾脏切片获得的不同病理样本中发现的不同形态学、免疫表型和免疫组化特征,使得区分两种非霍奇金B细胞淋巴瘤成为可能:一种是浸润脾脏的套细胞淋巴瘤,另一种是累及骨髓和外周血的边缘区淋巴瘤。由于在骨髓和脾脏中发现了相似的IgH基因重排,因此可以考虑肿瘤淋巴细胞起源相同,随后进行不同克隆选择的假说。
我们的病例强调了对同一患者不同解剖部位的同时标本进行检查的有用性以及脾切除术的相关诊断作用。