Table 1.

Shared and divergent characteristics of CLL and MCL

CharacteristicCLLMCL
Incidence*,4  5.1 0.8 
Hereditary susceptibility, 2.4- to 8.5-fold risk 2-fold risk 
Demographics1,4-7  Elderly patients 
Males > Females 
More common in Caucasians 
Less frequent in Asians 
Initial oncogenic events del(13q), del(11q), tri(12), other (?) t(11;14), SOX11 
Phenotype   
 SIg Dim IgM, IgD Strong IgM, IgD 
 CD5 
 CD20 Weak Strong 
 CD23 − 
 CD200 − 
Cytogenetic alterations del(13q); del(11q); del(17p) 
  t(11;14); del(9p); genomic instability 
Somatic mutations66,67,116  DNA damage response pathway (ATM, TP53
 NF-κB pathway 
 NOTCH1 NOTCH1/NOTCH2 
 SF3B1 and splicing machinery Chromatin modifiers 
 MYD88 CDKN2A del 
MicroRNA dysregulation167  miR-16-1, miR-29 a/b/c, miR-34a, miR-150, miR-155, miR-181 
 miR-15a, miR-21, miR-223, miR-650 miR-17-92, miR-20b, miR-142-3p 
CharacteristicCLLMCL
Incidence*,4  5.1 0.8 
Hereditary susceptibility, 2.4- to 8.5-fold risk 2-fold risk 
Demographics1,4-7  Elderly patients 
Males > Females 
More common in Caucasians 
Less frequent in Asians 
Initial oncogenic events del(13q), del(11q), tri(12), other (?) t(11;14), SOX11 
Phenotype   
 SIg Dim IgM, IgD Strong IgM, IgD 
 CD5 
 CD20 Weak Strong 
 CD23 − 
 CD200 − 
Cytogenetic alterations del(13q); del(11q); del(17p) 
  t(11;14); del(9p); genomic instability 
Somatic mutations66,67,116  DNA damage response pathway (ATM, TP53
 NF-κB pathway 
 NOTCH1 NOTCH1/NOTCH2 
 SF3B1 and splicing machinery Chromatin modifiers 
 MYD88 CDKN2A del 
MicroRNA dysregulation167  miR-16-1, miR-29 a/b/c, miR-34a, miR-150, miR-155, miR-181 
 miR-15a, miR-21, miR-223, miR-650 miR-17-92, miR-20b, miR-142-3p 

SIg, surface immunoglobulin.

*

Rates are per 100 000 and age adjusted to the US standard population;

Risk of first-degree relatives of CLL and MCL of developing CLL or non-Hodgkin lymphomas, respectively.

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