Table 8

Clinical and molecular characteristics of Richter syndrome patients

VariableCohort RS, no. (%)Collected RS, no. (%)
Patients 16 14 
Median age at diagnosis, y, n = 16, n = 10* 64 59 
Sex (male) 11 (69) 10 (71) 
Binet stage at diagnosis, n = 16, n = 9* 
    A 6 (38) 3 (33) 
    B 6 (38) 3 (33) 
    C 4 (25) 3 (33) 
CD38, n = 14, n = 8* 
    30% or more 11 (79) 6 (75) 
ZAP-70, n = 8, n = 2* 
    20% or more 1 (12.5) 1 (50) 
IgV gene mutation status, n = 16, n = 3* 
    UM, less than 2% 12 (75) 0 (0) 
Genomic aberrations, n = 14, n = 3* 
    Deletion 11 2 (14) 0 (0) 
    Deletion 17 3 (21) 3 (100) 
    Deletion 13 3 (21) 1 (33) 
    Trisomy 12 8 (57) 0 (0) 
    Normal 3 (21) 0 (0) 
CD38 genotypes, n = 16, n = 14* 
    CC 5 (31) 5 (36) 
    GC 9 (56) 7 (50) 
    GG 2 (13) 2 (14) 
CD38 allele frequencies, n = 16, n = 14* 
    C 0.59 0.61 
    G 0.41 0.39 
VariableCohort RS, no. (%)Collected RS, no. (%)
Patients 16 14 
Median age at diagnosis, y, n = 16, n = 10* 64 59 
Sex (male) 11 (69) 10 (71) 
Binet stage at diagnosis, n = 16, n = 9* 
    A 6 (38) 3 (33) 
    B 6 (38) 3 (33) 
    C 4 (25) 3 (33) 
CD38, n = 14, n = 8* 
    30% or more 11 (79) 6 (75) 
ZAP-70, n = 8, n = 2* 
    20% or more 1 (12.5) 1 (50) 
IgV gene mutation status, n = 16, n = 3* 
    UM, less than 2% 12 (75) 0 (0) 
Genomic aberrations, n = 14, n = 3* 
    Deletion 11 2 (14) 0 (0) 
    Deletion 17 3 (21) 3 (100) 
    Deletion 13 3 (21) 1 (33) 
    Trisomy 12 8 (57) 0 (0) 
    Normal 3 (21) 0 (0) 
CD38 genotypes, n = 16, n = 14* 
    CC 5 (31) 5 (36) 
    GC 9 (56) 7 (50) 
    GG 2 (13) 2 (14) 
CD38 allele frequencies, n = 16, n = 14* 
    C 0.59 0.61 
    G 0.41 0.39 

Percentages refer to total number of events scored.

UM indicates unmutated.

*

Number of events scored in the collected RS cohort.

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