The Outcome of Hemolytic Disease of the Fetus and Newborn Caused by Anti-Rh17 Antibody: Analysis of Three Cases and Review of the Literature

Abstract

Background: Anti-Rh17 is a rare red blood cell (RBC) antibody to high-frequency antigens that may cause severe hemolytic disease of the fetus and newborn (HDFN). Despite the rarity of HDFN caused by Anti-Rh17, this antibody was reported in many different populations. Emergency transfusions, especially exchange transfusions, present a huge problem if no compatible RBCs of phenotype D-- are available. Methods: Here we report obstetrical histories of three women and describe their pregnancies complicated by anti-Rh17 antibodies. We summarized published cases of pregnancies complicated by anti-Rh17 and reviewed transfusion treatment and outcomes. Additionally, a simplified flowchart for the management of such pregnancies is proposed. Results: Four pregnancies were affected by severe HDFN, and three of them ended with perinatal death. In the fourth case, the baby was born hydropic and icteric and the condition was rapidly deteriorating. Emergency exchange transfusion was performed with incompatible O-negative RBC units in AB-negative plasma. The baby was discharged on the 14th day in good health. In the available literature, 15 women and 22 pregnancies were reported, 20 of them developed severe HDFN. According to the data, intrauterine transfusion for treatment of HDFN was the most common form of treatment with the donation of the mother’s blood. Different options for exchange transfusion were described, including incompatible RBCs. Conclusion: In more than 90% of described pregnancies of HDFN caused by anti-Rh17 antibody, transfusion treatment was required. Therefore, RBC from D-- phenotype has to be available. According to published data, in emergent circumstances when maternal and blood from donor with phenotype D-- is not available, incompatible exchange transfusion is a better choice than delaying transfusion when it is necessary. It is of essential importance that pregnancies with high risk of HDFN due to anti-Rh17 are managed by a multidisciplinary team (transfusion medicine specialist, obstetrician, neonatologist) in a highly specialized tertiary institution.

Rh-D primigravida mother with anti Rh-17 antibodies causing mild haemolytic disease of foetus and newborn in baby: a case report

“…Any additional blood loss must be prevented including restricting the number and volume of blood draws during sample collection. In case of elective surgery, iron therapy with preoperative autologous collection as well as use of intraoperative red cell salvage and autologous/ allogeneic frozen red cell units are the good options available 1,5 .…”

“…Mother may be advised to take iron and folic acid supplementation or recombinant human erythropoietin and intravenous iron for the prevention of anaemia. It is mentioned that for foetal transfusion therapy, even ABO incompatible maternal blood are a good option because the fetus does not have its own anti-A and anti-B antibodies, so maternal red cells would not be hemolyzed 1,4 . For ET, ABO compatible maternal blood with the cord blood may be the best option if mother can tolerate blood donation 1 .…”

“…It is mentioned that for foetal transfusion therapy, even ABO incompatible maternal blood are a good option because the fetus does not have its own anti-A and anti-B antibodies, so maternal red cells would not be hemolyzed 1,4 . For ET, ABO compatible maternal blood with the cord blood may be the best option if mother can tolerate blood donation 1 . Other options for foetal IUT or ET are Rh-D-phenotype matched blood from mother's sibling or frozen rare Rh-D-donor blood 4,10,11,13 .…”

“…Here, two closely linked genes located on chromosome-1 control the expression of the Rh antigens. RHD gene codes for the D polypeptides while RHCE gene determines the C, c, E, e antigens 1 . Red blood cell alloimmunization against Rh antigen can occurs following sensitization by pregnancy or blood transfusion resulting in haemolytic transfusion reaction (HTR) and haemolytic disease of foetus and newborn (HDFN) 2 .…”

A null allele caused by a four‐base‐pair duplication within the RHCE gene encoding a D– – phenotype

“…Carriers can develop anti-Rh17, which may cause severe hemolytic disease of the fetus and newborn or fetal death in subsequent pregnancies. 1,2 Although the D− − phenotype is rare, it has been reported in various ethnicities. Deletions or insertions in the coding sequence of the RHCE gene can lead to frameshift variations and obliteration of the RhCE protein expression, causing the D− − phenotype.…”