PMID- 17325486 OWN - NLM STAT- MEDLINE DCOM- 20070426 LR - 20151119 IS - 0147-5185 (Print) IS - 0147-5185 (Linking) VI - 31 IP - 3 DP - 2007 Mar TI - Splenic marginal-zone lymphoma: one or more entities? A histologic, immunohistochemical, and molecular study of 42 cases. PG - 438-46 AB - 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. FAU - Papadaki, Theodora AU - Papadaki T AD - Hemopathology Department, Evangelismos Hospital, Athens, Greece. FAU - Stamatopoulos, Kostas AU - Stamatopoulos K FAU - Belessi, Chrysoula AU - Belessi C FAU - Pouliou, Evi AU - Pouliou E FAU - Parasi, Aikaterini AU - Parasi A FAU - Douka, Vassiliki AU - Douka V FAU - Laoutaris, Nikolaos AU - Laoutaris N FAU - Fassas, Athanasios AU - Fassas A FAU - Anagnostopoulos, Achilles AU - Anagnostopoulos A FAU - Anagnostou, Dimitra AU - Anagnostou D LA - eng PT - Journal Article PL - United States TA - Am J Surg Pathol JT - The American journal of surgical pathology JID - 7707904 RN - 0 (Biomarkers, Tumor) RN - 0 (DNA, Neoplasm) RN - 0 (Immunoglobulin Heavy Chains) RN - 0 (Immunoglobulin Light Chains) RN - 0 (RNA, Neoplasm) SB - IM MH - Biomarkers, Tumor/metabolism MH - DNA Mutational Analysis MH - DNA, Neoplasm/analysis MH - Gene Rearrangement, B-Lymphocyte, Heavy Chain/genetics MH - Gene Rearrangement, B-Lymphocyte, Light Chain/genetics MH - Humans MH - Immunoglobulin Heavy Chains/genetics MH - Immunoglobulin Light Chains/genetics MH - Immunophenotyping MH - Lymphoma/genetics/metabolism/*pathology MH - RNA, Neoplasm/analysis MH - Spleen/*pathology MH - Splenectomy MH - Splenic Neoplasms/genetics/metabolism/*pathology EDAT- 2007/02/28 09:00 MHDA- 2007/04/27 09:00 CRDT- 2007/02/28 09:00 PHST- 2007/02/28 09:00 [pubmed] PHST- 2007/04/27 09:00 [medline] PHST- 2007/02/28 09:00 [entrez] AID - 00000478-200703000-00013 [pii] AID - 10.1097/01.pas.0000213419.08009.b0 [doi] PST - ppublish SO - Am J Surg Pathol. 2007 Mar;31(3):438-46. doi: 10.1097/01.pas.0000213419.08009.b0.