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Published on: January 9, 2019
Defining the borders of splenic marginal zone lymphoma: a multiparameter study
Scott D Dufresne1, Raymond E Felgar, Rachel L Sargent
1Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
Human Pathology
|December 17, 2009
Summary
This study suggests including both biphasic and monophasic splenic marginal zone lymphomas, noting biologic differences in monophasic cases. A significant proportion of unclassifiable splenic lymphomas were identified, some with frequent +12 abnormalities.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Classic splenic marginal zone lymphoma (SMZL) is characterized by specific immunophenotypes and biphasic white pulp nodules.
- The 2008 WHO classification includes monophasic SMZL and unclassifiable splenic small B-cell lymphomas, necessitating further classification.
- Understanding the relationship between these entities is crucial for accurate diagnosis and treatment.
Purpose of the Study:
- To investigate the relationship between classic, monophasic, and unclassifiable splenic lymphomas.
- To analyze the immunophenotypic, clinical, and cytogenetic features of these splenic B-cell lymphomas.
- To refine the classification of splenic marginal zone lymphomas.
Main Methods:
- A multiparameter study was conducted on 47 cases of splenic marginal zone lymphomas and unclassifiable splenic small B-cell lymphomas.
- Immunophenotyping, fluorescence in situ hybridization (FISH), and classical cytogenetics were employed.
- Clinical data and survival rates were analyzed.
Main Results:
- Biphasic and monophasic cases were grouped as SMZL, with monophasic cases showing less typical phenotypes, often due to IgD negativity.
- Unclassifiable cases lacked a typical marginal zone component and showed distinct cytogenetic profiles, including frequent +12.
- CDK6 and BCL3 rearrangements were exclusively observed in the unclassifiable group.
Conclusions:
- Both biphasic and monophasic cases should be considered SMZL, but monophasic cases exhibit biologic differences.
- A substantial number of unclassifiable splenic B-cell lymphomas exist, with a subset frequently showing +12.
- Further research is needed to fully elucidate the spectrum of splenic small B-cell lymphomas.
