What Is Rh Null Blood Plus The Other

The clinical significance of its assessment is that such patients with Rhnull syndrome are associated with chronic hemolytic anemia of varying degrees.

Sorry AB-negative; youre not the only rare blood type in the world.

First discovered in an Aboriginal Australian woman in 1961, the Rh null ( Rhesus null ) is one of the rarest and most precious blood types in the world.

This stuff is characterized by a complete lack of antigens in the Rh system, which is the largest blood group system.

What Is Rh Null Blood Plus The Other

This includes the D antigen ( Rh factor, baby), plus the other 50-something antigens in the group.

His blood plasma is called Golden.

Rh-null blood.

After life-threatening lung surgery at age 14, which left him hospitalized for three months.

His life was saved thanks to transfusions of donated blood plasma.

However, because of Australias regulations for blood donations, he had to wait until he was 18.

It wasnt until a decade later it was discovered that his blood contained an important antibody which was needed to make Anti-D injections.

Harrisons blood plasma contains a rare antibody thats used to make a medication called anti-D immunoglobulin, also known as Rh immunoglobulin.

This medication is given to mothers who are at risk for something called Rh incompatibility with their fetus, which means the mothers immune system may attack and destroy the fetuss red blood cells.

Rhnull phenotype is a rare blood group characterized by the lack of expression of all Rh antigens (D, C, c, E and e) on the red cells.

The phenotype is further classified into the regulator and amorph type based on underlying genetic defect.

The clinical significance of its recognition is that such patients suffer from Rhnull syndrome associated with osmotically fragile red cells called stomatocytes with subsequent chronic haemolytic anaemia of varying degree.

Another importance is that such subjects readily form alloantibodies on exposure to Rh antigens.

The Rhnull phenotype is an inherited condition that may arise from homozygosity either for a suppressor gene unrelated to the RH locus (regulator type) or for a silent allele at the RH locus itself (amorph type).

A current model suggests that the proteins of the Rh complex (Rh, RhAG, CD47, LW, GPB) are assembled by non-covalent bonds and that it is not assembled or transported to the cell surface when one subunit is missing.

Rh and RhAG proteins belong to the same protein family and are quantitatively the major components that form the core of the complex, which is firmly linked to the membrane skeleton.

Molecular analysis of Rhnull individuals has revealed that abnormalities occur only at the RHAG and RH loci, without alteration of the genes encoding the accessory chains.

Mutations of the RHAG gene, but not of RH, occur in all Rhnull individuals of the regulator type (including Rhmod) investigated so far (13 cases), strongly suggesting that RHAG mutants act as suppressors and not as transcriptional regulators of the RH genes and that variable expression of the RHAG alleles may account for the Rhmod phenotypes (exhibiting weak expression of Rh antigens).

Conversely, mutations of the RHCE gene, but not of RHAG, occur in two unrelated Rhnull individuals of the amorph type, supporting the view that RH mutants result from a silent allele at the RH locus.

These findings strongly support the Rh complex model since when either the Rh or RhAG protein is missing, the assembly andor transport of the Rh complex is defective.

Transcriptional as well as post-transcriptional mechanisms may account for the molecular abnormalities, but experimental evidence based on expression models is required to test these hypotheses, in the hope that they may help to clarify the biological role of the Rh proteins in the red cell membrane.

It is characterized by the weak (Rhmod) or lack (Rhnull) of expression of all Rh antigens on the red cells.