Monoclonal B‐cell lymphocytosis in first‐degree relatives of patients with sporadic (non‐familial) chronic lymphocytic leukaemia

Matos, Daniel Mazza; Ismael, Sebastião José; Scrideli, Carlos Alberto; Oliveira, Fábio Morato de; Rego, Eduardo Magalhães; Falcão, Roberto Passetto

doi:10.1111/j.1365-2141.2009.07861.x

Cited by 36 publications

(48 citation statements)

References 31 publications

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“…MBL is more common in first-degree relatives of patients with familial CLL, with a reported prevalence of 12% to 18% [14–16]. This elevated MBL risk is particularly evident in young adults aged 16 to 40 years with a 17-fold relative risk, suggesting that there is an inherited abnormality that increases susceptibility to development of MBL at a much earlier age than the general population.…”

Section: Cll-like Mblmentioning

confidence: 99%

“…In relatives of patients with sporadic CLL the overall prevalence has been reported in the range of 4%, similar to that detected in the general population. However, in individuals aged more than 60 years from families with sporadic CLL, the MBL risk seems to be significantly increased (approximately 16%) similar to that seen in relatives of familial CLL cases [14]. …”

Section: Cll-like Mblmentioning

confidence: 99%

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New Insights into Monoclonal B-Cell Lymphocytosis

Kalpadakis

Pangalis

Sachanas

et al. 2014

BioMed Research International

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Monoclonal B-cell lymphocytosis (MBL) is a premalignant condition characterized by the presence of less than 5000/μL circulating clonal B cells in otherwise healthy individuals. Three subcategories have been identified according to the immunophenotypic features: CLL-like, CD5(+) atypical, and CD5(−) MBL. CLL-like MBL is by far the most frequent and best studied category and further divided in low-count [LC] and high-count [HC] MBL, based on a cutoff value of 500/μL clonal B cells. LC-MBL typically remains stable and probably does not represent a truly premalignant condition, but rather an age-related immune senescence. On the other hand, HC-MBL is closely related to CLL-Rai0, bearing similar immunogenetic profile, and is associated with an annual risk of progression to CLL requiring therapy at a rate of 1.1%. Currently there are no reproducible factors for evaluating the risk of progression to CLL. CD5(−) MBL is characterized by an immunophenotype consistent with marginal zone origin and displays many similarities with marginal zone lymphomas (MZL), mainly the splenic MZL. The cutoff value of 5000/μL clonal B cells cannot probably be applied in CD5(−) MBL, requiring a new definition to describe those cases.

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Section: Cll-like Mblmentioning

confidence: 99%

Section: Cll-like Mblmentioning

confidence: 99%

New Insights into Monoclonal B-Cell Lymphocytosis

Kalpadakis

Pangalis

Sachanas

et al. 2014

BioMed Research International

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“…24 Moreover, the Salamanca group found cll-type clones in more than 20 % individuals over 60 years of age by using eight-colour panels and acquired five million peripheral blood cells per individual. 25 The highest reported prevalence of cll-like MBl occurs in first-degree relatives of cll patients. Studies in both the uK and uSa showed a high prevalence of cll-like MBl (13.5-18 %) in individuals with a family history of cll who had normal blood counts.…”

Section: Diagnosismentioning

confidence: 99%

“…Studies in both the uK and uSa showed a high prevalence of cll-like MBl (13.5-18 %) in individuals with a family history of cll who had normal blood counts. 25,26 For instance, in the uK study, and using the same study methodology in both cll families and the general population, a fourfold increase prevalence of MBl in families with a genetic predisposition to cll was observed. …”

Section: Diagnosismentioning

confidence: 99%

New Developments in Chronic Lymphocytic Leukaemia Diagnosis

Delgado¹,

Santacruz²,

Baumann³

et al. 2013

European Oncology &Amp; Haematology

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Abstractchronic lymphocytic leukaemia (cll) is the most prevalent leukaemia in the developed world, with a remarkable racial and sex diversity.The diagnosis of cll is based on the presence in blood of more than 5,000 monoclonal B cells/µl, with a distinctive immunophenotype (cd5+, Smig dim , cd20 dim , cd19, cd23), which persists for more than 3 months. Newer monoclonal antibodies, such as anti-cd43, anti-cd200 or anti-RoR1, may provide further information in differentiating cll from other chronic lymphoproliferative disorders. cll lacks a specific genetic signature regarding chromosomic aberrations, but in cases of uncertain diagnosis molecular genetics including fluorescent in situ hybridisation may be useful. Next-generation sequencing techniques are unfolding the molecular heterogeneity of cll. in Europe, the incidence rate is similar (4.9 cases/year), with a similar sex distribution (5.9 for males and 4.0 for females). 5 The incidence of cll dramatically increases with age, reaching >20 cases/year in individuals older than 70, and even >30 cases/year in patients aged 80 or older.on the contrary, cll is infrequent in persons under the age of 40 (0.2 cases/year). 4 one of the most notorious epidemiological characteristics of cll is its racial diversity. Within the uS, the incidence rate is highest in caucasian males (6.2 cases/year) and females (3.2 cases/year) compared with african americans (4.3 and 2.0 cases/year for males and females, respectively), while asian americans and Pacific islanders had the lowest incidence (1.3 and 0.7 cases/year for males and females, respectively). 4incidence rates are even lower in the Far East, and countries such as Japan have an age-adjusted incidence of 0.48 cases/year, which is even lower than that of mantle-cell lymphoma (Mcl) in that region. 6Furthermore, the clinical outcome of patients with cll is also different across various ethnic groups. in the uS, african american patients with cll present at a younger age and with more frequent advanced disease, extranodal involvement and B symptoms compared with caucasian patients. consequently, african american patients had a significantly shorter event-free and overall survival, an effect that was found to be independent from other prognostic factors in a multivariable analysis. 7 By contrast, in a recent series or Japanese patients, most cases had favourable prognostic markers and an excellent clinical outcome. 8 it is important to note, however, that rather than diversity in the characteristics of the disease, these differences could reflect disparities in access to medical care. 7 DiagnosisThe hallmark of the disease is an increased white blood cell count with a high percentage of small, mature-looking lymphocytes. in patients diagnosed on routine analysis, anaemia is found in less than 10 % of the patients. importantly, anaemia is not always due to the infiltration of the bone marrow by the disease; other causes such as autoimmunity, iron, folic acid or vitamin B12 deficiency may account for the presence of anaemia a...

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“…7 Indeed, MBL is more frequent in sibling transplant donors to CLL patients (15.4%) than in the general population (3%), and MBL prevalence increases with age in first-degree relatives of patients with sporadic CLL. 8,9 In conclusion, we propose the use of sensitive methods for the systematic screening of blood donors for the presence of MBL, particularly when a history of CLL is known in the family. 10 …”

mentioning

confidence: 99%