Clone Emergence and Evolution in Chronic Lymphocytic Leukemia: Characterization of Clinical, Laboratory and Immunophenotypic Profiles of 25 Patients

Faguet, Guy B.; Agee, Julia F.; Marti, Gerald E.

doi:10.3109/10428199209053566

Cited by 15 publications

(9 citation statements)

References 29 publications

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“…This finding is similar to an earlier study of patients with mild lymphocytosis, eight out of 24 of whom progressed to CLL in 5 years [45]. The Leeds group defined MBL as an absolute B-cell count of less than 5.0 × 10 9 /l with no lymphadenopathy and less than 30% CLL cells in the bone marrow if assessed [42].…”

Section: Monoclonal B-cell Lymphocytosis: Precursor Condition?supporting

confidence: 88%

Inherited predisposition to chronic lymphocytic leukemia

Brown¹

2008

Expert Review of Hematology

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Inherited susceptibility to chronic lymphocytic leukemia (CLL) has been recognized for decades. Approximately 10% of individuals with CLL report a family history of CLL or a related lymphoproliferative disorder, and genetic predisposition is the best understood risk factor for CLL. Studies of familial CLL have suggested that the disease features are largely similar to sporadic CLL, although recent data suggest that familial CLL may more commonly show somatic hypermutation of the immunoglobulin heavy-chain variable region, suggesting a more indolent disease course. Monoclonal B-cell lymphocytosis (MBL) has been identified recently as a likely precursor to CLL; it is found in the general population with increasing age and enriched in unaffected relatives of individuals with familial CLL. Studies of MBL as well as mouse models of CLL may lead to better understanding of early CLL pathogenesis that is relevant to familial predisposition. To date, the identification of genes that predispose to familial CLL has been slow, primarily due to the relatively few families available for study, the small size of those families and disease causation most likely by multiple genes that each confer smaller risks. In the coming years, the application of systematic genomics approaches to familial CLL should, hopefully, lead to the identification of novel loci involved in the disease.

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Section: Monoclonal B-cell Lymphocytosis: Precursor Condition?supporting

confidence: 88%

Inherited predisposition to chronic lymphocytic leukemia

Brown¹

2008

Expert Review of Hematology

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“…This is in keeping with the finding that a large proportion of patients with early stage CLL show stable disease and, occasionally, decreasing CLL cell counts (Table I). Faguet et al (1992) reported a series of 39 patients with a slight increase in their absolute lymphocyte count; 24 had an abnormal B-cell clone and eight of the 24 patients progressed to clinical B-cell CLL over 5 years. In the limited NCI follow-up of familial MBL, progression to CLL has not yet been observed.…”

Section: Natural History Of the Disordermentioning

confidence: 99%

Diagnostic criteria for monoclonal B‐cell lymphocytosis

et al. 2005

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SummaryVery low levels of circulating monoclonal B‐cell subpopulations can now be detected in apparently healthy individuals using flow cytometry. We propose the term ‘monoclonal B‐cell lymphocytosis’ (MBL) to describe this finding. The aim of this document is to provide a working definition of MBL for future clinical, epidemiological and laboratory studies. We propose that the detection of a monoclonal B‐cell population by light chain restriction is sufficient to define this condition in individuals not meeting the diagnostic criteria for other B‐lymphoproliferative disorders. The majority of individuals with MBL will have cells that are indistinguishable from chronic lymphocytic leukaemia (CLL). However, this blood cell clonal expansion of CD5+ or CD5− B‐lymphocytes is age‐dependent and immunophenotypic heterogeneity is common. Longitudinal studies are required to determine whether MBL is a precursor state to CLL or other B‐lymphoproliferative disease in a situation analogous to a monoclonal gammopathy of undetermined significance and myeloma. Future studies of MBL should be directed towards determining its relationship to clinical disease, particularly in individuals from families with a genetic predisposition to developing CLL.

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“…However, the amount of data documenting the clinical course and outcome of MBL has not kept pace with the number of new diagnoses [6]. There is little information describing the clinical course of MBL, its prognostic factors or characteristics, and its relationship to CLL [10]. Here we present a retrospective study of 214 patients with clonal lymphoproliferation in keeping with CLL, 46 of whom presented with MBL.…”

Section: Introductionmentioning

confidence: 96%

Clinical progression and outcome of patients with monoclonal B-cell lymphocytosis

Fung

Hillier

Leger

et al. 2007

Leukemia & Lymphoma

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Monoclonal B-cell lymphocytosis (MBL) is a clonal lymphoproliferation with the immunophenotype of chronic lymphocytic leukemia (CLL) but a B-lymphocyte count of less than 5 x 10(9)/l and no lymphadenopathy, organomegaly, cytopenias or symptoms. We performed a retrospective analysis of patients with MBL (n = 46), Rai stage 0 CLL (n = 112) and Rai stage > or =1 CLL (n = 54). Median follow-up and range was 30 (0.1-120) months for MBL, 60 (0.1-309) months for stage 0 CLL and 54 (0.1-309) months for stage > or =1 CLL. None of the MBL patients required treatment compared with 24 of 112 (21%) stage 0 CLL and 28 of 54 (52%) stage > or =1 CLL patients (p < 0.0003). No MBL underwent aggressive transformation compared with 1 of 112 (0.8%) stage 0 CLL and 6 of 54 (11%) stage > or =1 CLL patients (p < 0.0003). Progression-free survival (PFS) appeared improved in MBL compared to stage 0 CLL, although this did not reach statistical significant (p = 0.07) due to the relatively short follow-up in the MBL group; two year PFS was 97.2% for MBL, 93.1% for stage 0 CLL, and 68% for stage > or =1 CLL patients (p < 0.0001 for stage > or =1 CLL compared with MBL and stage 0 CLL). This is the first study of outcome in MBL which demonstrates that patients have an improved disease course compared to stage 0 CLL patients. Over a median 2.5 years of follow-up, no MBL patients required treatment or died of CLL-related causes.

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Clone Emergence and Evolution in Chronic Lymphocytic Leukemia: Characterization of Clinical, Laboratory and Immunophenotypic Profiles of 25 Patients

Cited by 15 publications

References 29 publications

Inherited predisposition to chronic lymphocytic leukemia

Inherited predisposition to chronic lymphocytic leukemia

Diagnostic criteria for monoclonal B‐cell lymphocytosis

Clinical progression and outcome of patients with monoclonal B-cell lymphocytosis

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