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Title: Lymphoma-like monoclonal B cell lymphocytosis in a patient population: biology, natural evolution, and differences from CLL-like clones
Authors: Vander Meeren, Sam
Heyrman, Bert
Renmans, Wim
Bakkus, Marleen
MAES, Brigitte 
De Raeve, Hendrik
Schots, Rik
Jochmans, Kristin
Issue Date: 2018
Publisher: SPRINGER
Source: ANNALS OF HEMATOLOGY, 97(7), p. 1219-1227
Abstract: High-count monoclonal B cell lymphocytosis (MBL) with a chronic lymphocytic leukemia (CLL) phenotype is a well-known entity, featuring 1-4% annual risk of progression towards CLL requiring treatment. Lymphoma-like MBL (L-MBL), on the other hand, remains poorly defined and data regarding outcome are lacking. We retrospectively evaluated 33 L-MBL cases within our hospital population and compared them to 95 subjects with CLL-like MBL (C-MBL). Diagnoses of L-MBL were based on asymptomatic B cell clones with Matutes score < 3, B cells < 5.0 x 10(3)/mu l, and negative computerized tomography scans. We found that median B cell counts were considerably lower compared to C-MBL (0.6 vs 2.3 x 10(3)/mu l) and remained stable over time. Based on immunophenotyping and immunogenetic profiling, most L-MBL clones did not correspond to known lymphoma entities. A strikingly high occurrence of paraproteinemia (48%), hypogammaglobulinemia (45%), and biclonality (21%) was seen; these incidences being significantly higher than in C-MBL (17, 21, and 5%, respectively). Unrelated monoclonal gammopathy of undetermined significance was a frequent feature, as the light chain type of 5/12 paraproteins detected was different from the clonal surface immunoglobulin. After 46-month median follow-up, 2/24 patients (8%) had progressed towards indolent lymphoma requiring no treatment. In contrast, 41% of C-MBL cases evolved to CLL and 17% required treatment. We conclude that clinical L-MBL is characterized by pronounced immune dysregulation and very slow or absent progression, clearly separating it from its CLL-like counterpart.
Notes: [Vander Meeren, Sam; Renmans, Wim; Bakkus, Marleen; Jochmans, Kristin] Vrije Univ Brussel, Univ Ziekenhuis Brussel, Div Hematol, Dept Biol Clin, Brussels, Belgium. [Heyrman, Bert] ZNA Middelheim, Div Hematol, Dept Internal Med, Antwerp, Belgium. [Maes, Brigitte] Jessa Ziekenhuis, Div Hematol, Dept Clin Biol, Hasselt, Belgium. [De Raeve, Hendrik] Vrije Univ Brussel, Univ Ziekenhuis Brussel, Dept Pathol, Brussels, Belgium. [Schots, Rik] Vrije Univ Brussel, Univ Ziekenhuis Brussel, Div Hematol, Dept Internal Med, Brussels, Belgium.
Keywords: Monoclonal B cell lymphocytosis; Chronic lymphocytic leukemia; Non-Hodgkin's lymphoma; MGUS; Immunophenotyping
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ISSN: 0939-5555
e-ISSN: 1432-0584
DOI: 10.1007/s00277-018-3282-0
ISI #: 000433502600007
Category: A1
Type: Journal Contribution
Appears in Collections:Research publications

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