Table 2

Complement system analysis in patients with HSCT-TMA

PatientTransplant typeCFI,CFH,MCP,CFB,CFR5 (direct sequence analysis)Recipient CFH-CFHR5 (MLPA)Donor CFH-CFHR5 (MLPA)CFH antibody (ELISA)CFHR1 protein analysis (western blot)
autologous normal alleles *del(CFHR3-CFHR1) n/a absent present 
autologous normal alleles *del(CFHR3-CFHR1) n/a absent present 
autologous normal alleles *del(CFHR1-CFHR4) n/a absent present 
allogeneic normal alleles *del(CFHR3-CFHR1) normal allele present present 
allogeneic normal alleles *del(CFHR3-CFHR1) *del(CFHR3-CFHR1) present present 
allogeneic normal alleles normal allele normal allele present present 
PatientTransplant typeCFI,CFH,MCP,CFB,CFR5 (direct sequence analysis)Recipient CFH-CFHR5 (MLPA)Donor CFH-CFHR5 (MLPA)CFH antibody (ELISA)CFHR1 protein analysis (western blot)
autologous normal alleles *del(CFHR3-CFHR1) n/a absent present 
autologous normal alleles *del(CFHR3-CFHR1) n/a absent present 
autologous normal alleles *del(CFHR1-CFHR4) n/a absent present 
allogeneic normal alleles *del(CFHR3-CFHR1) normal allele present present 
allogeneic normal alleles *del(CFHR3-CFHR1) *del(CFHR3-CFHR1) present present 
allogeneic normal alleles normal allele normal allele present present 

CFR, complement factor H-related gene 5.

*

del refers to heterozygous deletions.

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