Defining the borders of splenic marginal zone lymphoma: a multiparameter study

Dufresne, Scott D.; Felgar, Raymond E.; Sargent, Rachel L.; Surti, Urvashi; Gollin, Susanne M.; McPhail, Ellen D.; Cook, James R.; Swerdlow, Steven H.

doi:10.1016/j.humpath.2009.09.007

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Volume 41, Issue 4 , Pages 540-551, April 2010

Defining the borders of splenic marginal zone lymphoma: a multiparameter study

Scott D. Dufresne, MD
- Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
Raymond E. Felgar, MD, PhD
- Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
Rachel L. Sargent, MD
- Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
Urvashi Surti, PhD
- Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
- Pittsburgh Cytogenetics Laboratory, Magee-Womens Hospital of UPMC, Pittsburgh, PA 15213, USA
Susanne M. Gollin, PhD
- Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
- Pittsburgh Cytogenetics Laboratory, Magee-Womens Hospital of UPMC, Pittsburgh, PA 15213, USA
- Department of Human Genetics, University of Pittsburgh Graduate School of Public Health and University of Pittsburgh Cancer Institute Cytogenetics Facility, Pittsburgh, PA 15213, USA
Ellen D. McPhail, MD
- Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 53902, USA
James R. Cook, MD, PhD
- Department of Clinical Pathology, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
Steven H. Swerdlow, MD
- Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
- Corresponding author. Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, UPMC-Presbyterian Hospital, Pittsburgh, PA 15213, USA.

Received 26 June 2009; received in revised form 12 September 2009; accepted 18 September 2009. published online 11 December 2009.

Summary

Classic splenic marginal zone lymphomas are CD5−, CD10−, CD23−, CD43−, and usually IgD+ with biphasic white pulp nodules. However, the 2008 World Health Organization classification accepts splenic marginal zone lymphomas with monophasic marginal zone-like white pulp nodules and recognizes a group of unclassifiable splenic small B-cell lymphomas. To explore the relationship of classic splenic marginal zone lymphomas to these other less well-defined splenic lymphomas, a multiparameter study of 47 splenic marginal zone lymphomas and unclassifiable splenic small B-cell lymphomas was performed. Seventeen of 31 splenic marginal zone lymphomas were biphasic, and 14 were monophasic (90%-100% marginal zone-like white pulp nodules). Sixteen cases were unclassifiable splenic small B-cell lymphomas, most lacking a marginal zone-type component. There were many clinical similarities between the 3 groups, including similar survivals. Monophasic and unclassifiable cases were less likely to have a typical splenic marginal zone lymphoma phenotype (28.6%, 23.1%) compared with biphasic cases (86.7%), usually because of IgD negativity (P < .003). Thirty-four of 42 (81%) cases had cytogenetic abnormalities by fluorescence in situ hybridization; and 17 of 20 (85%), by classical cytogenetics. The most frequent fluorescence in situ hybridization abnormalities among the splenic marginal zone lymphomas were del(7)(q31) (26%), +12 (25%), and +3q27 (27%); and among the unclassifiable cases, +12 (50%) and +3q27 (36%). Five of 6 unclassifiable cases with exclusively small non-marginal zone-like lymphocytes involving both white and red pulp had +12 compared with 9 of 34 other cases (P < .02). CDK6 (2 cases) and BCL3 (1 case) rearrangements were only seen in the unclassifiable group. These results support including both biphasic and monophasic cases as splenic marginal zone lymphomas, but suggest that the lack of a non-marginal zone-like population in the monophasic group is associated with some biologic differences. They also demonstrate a relatively large proportion of unclassifiable cases, including a group with frequent +12.

Keywords: Splenic marginal zone lymphoma, Spleen, B-Cell lymphoma, Marginal zone lymphoma, Fluorescence in situ hybridization