Maternal red blood cell alloimmunisation Working Party, literature review. RH blood group system: Rare specificities.

This report is part of a series reporting the GRADE review performed by the 2018-2020 French Working Party on maternal red blood cell alloimmunisation. This report focusses on the clinical significance in obstetrics, as published in the scientific literature, of the rare RH antibodies, variants and antigens (i.e. excluding conventional RH1 trough RH8 antigens, RH12, RH22 and RH27, which are discussed in other reports of this series). Extremely severe or severe haemolytic disease of the fetus and the newborn (HDFN), leading to death or requiring transfusions, have been reported for: anti-RH1 (-D) associated with DVI, DBT and DIVb phenotypes, RHD*12.04 (DOL4), RHD*03.03 (DIIIc), RHD*D-CE(2-5)-D, RHD*01EL.31 (RHD*148+1T), anti-RH9 (-CX), anti-RH11 (-EW), anti-RH17 (-Hr0), anti-RH18 (-Hr), anti-RH19 (-hrS), anti-RH23 (-DW), anti-RH29 ("total" Rh), anti-RH30 (-Goa), anti-RH32, anti-RH34 (-HrB), anti-RH36 (-Bea), anti-RH40 (-Tar), anti-RH46 (-Sec), anti-RH48 (-JAL), anti-RH54 (DAK), and antibodies to high prevalence antigens such as those associated with RHCE*02.08.02 (RHCE*CW-RHD(6-10)), RHCE*03N.01 (RHCE*cEMI). HDFN of moderate, mild or undetailed severity have been reported for: anti-RH1 associated with DHar, DIIIa and DIVa phenotypes, RHD*01EL.08 (RHD*486+1A),RHD*01EL.44 (RHD*D-CE(4-9)-D),RHD*25 (DNB), anti-RH20 (-VS), anti-RH31 (-hrB), anti-RH37 (-Evans), ani-RH42, anti-RH49 (-STEM), anti-RH51 (-MAR), anti-RH55 (-LOCR), anti-RH58 (-CELO). Positive direct antiglobulin test in the newborn but no clinically significant HDFN has been reported for anti-RH1 (-D) associated with RHD*10.05 (DAU5), RHD*12.02 (DOL2). Because so many specificities are associated with severe HDFN in the RH system, all RH antibodies should be considered as potentially able to cause HDFN, even if none has been reported yet.