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Clonal B-Cell Lymphocytosis of Marginal Zone Origin: Presenting Features, Clinical Evolution and Prognostic Factors
Sotirios Sachanas1, Gerassimos A Pangalis1, Christina Kalpadakis2
1Department of Haematology, Athens Medical Center-Psychiko Branch, 11525 Athens, Greece.
Cancers
|July 15, 2026
Summary
Clonal B-cell lymphocytosis of marginal zone origin (CBL-MZ) is an indolent disorder with a good prognosis and low progression risk. Bone marrow infiltration and elevated LDH are key indicators for initiating therapy.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Circulating clonal B-cells with marginal zone (MZ) features have been described under various terms.
- Understanding the characteristics and prognosis of clonal B-cell lymphocytosis of marginal zone origin (CBL-MZ) is crucial.
Purpose of the Study:
- To elucidate the main disease characteristics of CBL-MZ.
- To identify prognostic factors for patient outcomes in CBL-MZ.
Main Methods:
- Multicenter retrospective analysis of 98 consecutive CD5(-) CBL cases.
- Inclusion criteria: circulating CD5(-) clonal B-cells without other lymphoproliferative disorder features.
- Comprehensive analysis of clinical, morphologic, immunophenotypic, biochemical, histologic, and molecular data.
Main Results:
- Two CBL-MZ subcategories identified: one with paraproteinemia and MYD-88L265P mutation, another with a leukemic picture and CD11c expression.
- Median treatment-free survival (TFS) was 91%, with median freedom from progression (FFP) of 95.6 months.
- Bone marrow (BM) infiltration ≥ 50% and elevated lactate dehydrogenase (LDH) were significant predictors for TFS.
Conclusions:
- CBL-MZ is an indolent lymphoproliferative disorder with an excellent prognosis and low progression rate.
- The disease is heterogeneous, with most cases remaining stable or showing increased lymphocytosis.
- BM infiltration extent and elevated LDH levels are critical indicators for therapy initiation.
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