Papadaki Theodora, Stamatopoulos Kostas, Belessi Chrysoula, Pouliou Evi, Parasi Aikaterini, Douka Vassiliki, Laoutaris Nikolaos, Fassas Athanasios, Anagnostopoulos Achilles, Anagnostou Dimitra
Hemopathology Department, Evangelismos Hospital, Athens, Greece.
Am J Surg Pathol. 2007 Mar;31(3):438-46. doi: 10.1097/01.pas.0000213419.08009.b0.
We analyzed 42 splenic marginal-zone lymphoma (SMZL) cases diagnosed on splenectomy specimens after established World Health Organization criteria. A predominantly nodular growth pattern was observed in 24 cases; the remainder showed predominantly (11/42) or exclusively (7/42) diffuse infiltration. Twenty-one cases showed the "classic" biphasic appearance; 13 cases exhibited marginal-zone morphology; finally, 8 cases were composed predominantly of small cells. CD21 and CD35 were expressed by 12/42 and 17/38 cases, respectively. DBA.44 was detected in 24/42 cases. Seventeen of 37 cases were surface IgD (SIgD)-positive. Twenty-one of 22 analyzed cases were SIgM-positive (12/21 coexpressed SIgD). Five of 37 cases were SIgG-positive. CD27 staining was observed in 21/35 cases; 7/18 CD27-positive cases coexpressed SIgD; 7/14 CD27-negative cases were SIgD-positive. Forty IGHV-D-J rearrangements were amplified in 34/42 cases: the IGHV4-34 gene predominated, followed by IGHV1-2. Using the 98% homology cut-off, 25/40 (62.5%) IGHV sequences were considered as "mutated": 10/11 cases with monomorphous, marginal-zone morphology were IGHV-mutated; in contrast, 4/6 cases with monomorphous, small-cell morphology were IGHV-unmutated. Five of 7 cases expressing IGHV1 subgroup genes had biphasic morphology, whereas 6/9 IGHV3-expressing cases had monomorphous, marginal-zone morphology. Most IGHV-mutated cases (14/20; 70%) were SIgD-negative; in contrast, 8/11 IGHV-unmutated cases expressed SIgD. CD27 was detected in 10/17 IGHV-mutated and 6/10 IGHV-unmutated cases. Seven of 11 CD27-negative cases were IGHV-mutated; 5/7 CD27-negative/IGHV-mutated cases expressed DBA.44. These results confirm the considerable histologic, immunohistochemical, and molecular heterogeneity of SMZL and indicate an origin from the diverse resident B-cell populations of the normal SMZ.
我们根据既定的世界卫生组织标准，对42例经脾切除术标本诊断的脾边缘区淋巴瘤（SMZL）病例进行了分析。24例观察到主要为结节状生长模式；其余病例主要（11/42）或仅（7/42）表现为弥漫性浸润。21例呈现“经典”的双相外观；13例表现为边缘区形态；最后，8例主要由小细胞组成。42例中有12例表达CD21，38例中有17例表达CD35。42例中有24例检测到DBA.44。37例中有17例表面IgD（SIgD）阳性。22例分析病例中有21例SIgM阳性（21例中有12例同时表达SIgD）。37例中有5例SIgG阳性。35例中有21例观察到CD27染色；18例CD27阳性病例中有7例同时表达SIgD；14例CD27阴性病例中有7例SIgD阳性。42例中有34例扩增出40个IGHV - D - J重排：IGHV4 - 34基因占主导，其次是IGHV1 - 2。使用98%同源性截断值，40个IGHV序列中有25个（62.5%）被认为是“突变的”：11例单形性边缘区形态病例中有10例IGHV突变；相比之下，6例单形性小细胞形态病例中有4例IGHV未突变。7例表达IGHV1亚组基因的病例中有5例具有双相形态，而9例表达IGHV3的病例中有6例具有单形性边缘区形态。大多数IGHV突变病例（20例中有14例；70%）SIgD阴性；相比之下，11例IGHV未突变病例中有8例表达SIgD。17例IGHV突变病例中有10例检测到CD27，10例IGHV未突变病例中有6例检测到CD27。11例CD27阴性病例中有7例IGHV突变；7例CD27阴性/IGHV突变病例中有5例表达DBA.44。这些结果证实了SMZL在组织学、免疫组织化学和分子水平上存在相当大的异质性，并表明其起源于正常脾边缘区不同的常驻B细胞群体。
