Table 1.

Summary of patient characteristics, n = 52

Age at investigation  
Median, y (range) 18 (2-65) 
Sex, n (%)  
Female 32 (62) 
Male 20 (38) 
Ethnicity, n (%)  
British Indian 1 (2) 
British Pakistani 4 (8) 
Druze 1 (2) 
East African 3 (6) 
Fijian Indian 1 (2) 
Finnish 23 (44) 
North African 1 (2) 
Puerto Rican 1 (2) 
Swedish 2 (4) 
White (not specified) 15 (28) 
Initial condition, n (%)  
Sibling with biallelic ERCC6L2 mutation without diagnosis of BMF, MDS, or AML 5 (10) 
BMF 32 (62) 
MDS 9 (17) 
MDS/AML  2 (4) 
AML 3 (6) 
Other HM (ALL) 1 (2) 
ERCC6L2 genotype, n (%)  
Homozygous 39 (75) 
Compound heterozygous 13 (25) 
Individuals by ERCC6L2 mutation type, n (%)  
Biallelic frameshift 29 (55) 
Biallelic nonsense 5 (10) 
Biallelic splicing 1 (2) 
Biallelic exon deletion 4 (8) 
Biallelic missense 4 (8) 
Mixed 9 (17) 
Age at investigation  
Median, y (range) 18 (2-65) 
Sex, n (%)  
Female 32 (62) 
Male 20 (38) 
Ethnicity, n (%)  
British Indian 1 (2) 
British Pakistani 4 (8) 
Druze 1 (2) 
East African 3 (6) 
Fijian Indian 1 (2) 
Finnish 23 (44) 
North African 1 (2) 
Puerto Rican 1 (2) 
Swedish 2 (4) 
White (not specified) 15 (28) 
Initial condition, n (%)  
Sibling with biallelic ERCC6L2 mutation without diagnosis of BMF, MDS, or AML 5 (10) 
BMF 32 (62) 
MDS 9 (17) 
MDS/AML  2 (4) 
AML 3 (6) 
Other HM (ALL) 1 (2) 
ERCC6L2 genotype, n (%)  
Homozygous 39 (75) 
Compound heterozygous 13 (25) 
Individuals by ERCC6L2 mutation type, n (%)  
Biallelic frameshift 29 (55) 
Biallelic nonsense 5 (10) 
Biallelic splicing 1 (2) 
Biallelic exon deletion 4 (8) 
Biallelic missense 4 (8) 
Mixed 9 (17) 

Per the ELN2022 classification.21 

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