Year : 2020 | Volume
: 5 | Issue : 1 | Page : 90--92
Case series of severe fetal hemolysis in Rh-C-negative mother
Toshniwal Pramod1, Nishanth Rajan1, Simon Kingsley2, Lalitha Krishnan1,
1 Department of Paediatrics, Pondicherry Institute of Medical Sciences, Puducherry, India
2 Department of Pathology, Pondicherry Institute of Medical Sciences, Puducherry, India
Department of Pathology, Pondicherry Institute of Medical Sciences, Puducherry
Anti-Rh(c) antibody is the third most common cause of severe hemolytic disease of the fetus and newborn (HDFN). The widespread use of anti-D immunoglobulin has led to arelative increase in the importance of non-RhD isoimmunization as a cause of HDFN. The Rhc is a part of the Rh blood group system and its prevalence in India is 52%–62%. Here, we report two cases with anti-c antibody with severe hyperbilirubinemia who required intravenous immunoglobulin, red blood cells transfusion, and phototherapy.
|How to cite this article:|
Pramod T, Rajan N, Kingsley S, Krishnan L. Case series of severe fetal hemolysis in Rh-C-negative mother.Glob J Transfus Med 2020;5:90-92
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Pramod T, Rajan N, Kingsley S, Krishnan L. Case series of severe fetal hemolysis in Rh-C-negative mother. Glob J Transfus Med [serial online] 2020 [cited 2021 May 17 ];5:90-92
Available from: https://www.gjtmonline.com/text.asp?2020/5/1/90/282742
Hemolytic disease of the fetus and newborn (HDFN) leads to severe degrees of fetal hemolysis and can result in fetal hydrops. Anit-Rh (D) antibody is a major cause of HDFN. After worldwide adoption of antenatal Rhesus immunoglobulin, its prevalence has been reducing since 1968. There are other red blood cell (RBC) antigens for which the maternal alloimmunization remains the cause for hemolytic disease of the newborn and there is no prophylactic immunoglobulin available. There are cases reported with mild-to-severe hemolytic disease with anti-c, C, e, E or Kell, Kidd, and Duffy antibodies, and these antibodies are detected in mother's serum. Diagnosis of this rare antigens gets delayed in hospitals and clinics in India and other developing nations because routine screening is done only for Rh (D)-negative mothers. In India, the first case with anti-c antibodies-mediated hemolytic disease was reported in 2007. Here, we are report two cases of anti-c antibodies-mediated significant hemolysis in newborns.
A 58-h-old term female baby born to a 37-year-old mother (G3P1 L1A1) by elective lower segment cesarean section elsewhere developed clinical jaundice at 12 h of life. The baby was treated with phototherapy, but as jaundice was progressing, she was referred to our institute for further management. On admission, total serum bilirubin (TSB) was 32.2 mg/dl, reticulocyte count 8.8%, and hematocrit was 34.2%.
The mother and baby blood group was found to be B Positive. Peripheral smear showed features of hemolysis. The baby's direct Coombs test (DCT) was positive (1+). The mother's indirect Coomb's test was positive (4+). The father's blood group was O positive. On further evaluation, it was found that the mother had anti-c antibodies and the baby and father had c antigen. Anti-c antibody titer was done with the father's red cells and found to be 1:64. The baby was treated with intravenous immunoglobulin (IVIG 1 g/kg over 4 h) and high-intensive phototherapy for 5 days, and for anemia, packed red cell transfusion (red cell concentrates) was given twice, on day 9 and 18 of life. The baby was shifted to the ward at day 16 of life with acceptable bilirubin and hemoglobin levels.
A 15-h-old neonate born to 26-year-old G4P1 L2A1 at 37 week + 4 days period of gestation, by normal vaginal delivery, was referred with hyperbilirubinemia. At admission, blood investigation revealed that TSB was 20.8 mg/dl and direct bilirubin was 1.3 mg/dl, complete blood count showed Hb of 8.6 g/dl with increased reticulocyte count (9.1%), and peripheral smear showed features of hemolytic anemia. The mother and her newborn's blood group was positive. DCT of the baby was positive (2+) and indirect Coombs test (ICT) of the mother was positive (4+). Hence, minor blood group incompatibility was suspected. On further evaluation, it was found that the mother had anti-c antibodies and the baby and father had c antigen. Anti-c antibody titer was done with father's red cells and found to be 1:16. Newborn was started on phototherapy and IVIG transfusion (1 g/kg) and packed cell transfusion (15 ml/kg). After 45 h of phototherapy, hemoglobin was 13.5 g/dl, and TSB was 10.9 mg/dl. Phototherapy was stopped. On day 5 of life, the baby was looking icteric, and on repeat TSB, it was 20.4 mg/dl, and phototherapy was restarted and given for 48 h.
The Rh blood group system is one of the most complex and immunogenic systems that produces hemolytic disease of the fetus and newborn. It is comprised at least 45 independent antigens, the most important of which are D, C, c, E, and e. These antigens are encoded by the RhD and RHCE genes, located together on chromosome1. The role of RhD antibody causing hemolytic disease of newborn is well known. Antibody c has a similar mechanism of causing hemolysis like antibody D. The prevalence of Rh-c antigen in the Indian population is 52%–62%. Anti-c antibodies prevalence among alloantibodies detected in patients has been reported to range from 3.1% to 17.5% in India.
Both our cases were DCT positive, case one DCT (1+) and case two DCT (2+). This may be due to the increased c antigenic expression in the second case. If the father has heterozygous c antigen, there is a 50% chance of the fetus having c antigen. If it is homozygous, there is a 100% chance of the fetus having the c antigen. This zygosity expression of c antigen may be responsible for DCT positivity. Our first case developed severe hemolysis compared to the second case. This is because of the high IgG anti-c antibody titer in case one compared to case two (1:64 vs. 1:16). Hackney et al. found that antibody c antibody titer of 1:32 was more associated with significant hemolysis and hydrops fetalis. Zaman et al. reported a case with a 1:4 titer of anti-c antibody managed with intrauterine transfusion.
The prevalence of anti-c antibodies in the Indian population was found to be 6.4%. In our study, both the newborns developed significant hyperbilirubinemia and required phototherapy, IVIG, and RBC transfusion. The first baby had antibody titer of 1:64 and had prolonged jaundice requiring phototherapy for 5 days and the second newborn had 1:16 anti-c antibody titer requiring phototherapy for 45 h.
Generally, ICT is done in all Rh-negative mothers during the antenatal period. Most of the time ICT to the mother and DCT to the baby will be done, after the newborn developed severe hemolytic disease. In our case series, both the mothers were Rh positive and not done ICT in antenatal periods. This will lead to increase the mortality and morbidity to the baby and mother. Routine ICT or antibody screening should be done in both Rh-positive and Rh-negative pregnant mothers to prevent perinatal mortality and minimize the morbidity. Knowing clinically significant IgG antibodies in the antenatal period will help to manage the fetus and newborn appropriately. It is a challenge to get an antigen-c-negative blood than the D antigen-negative blood for transfusion to the baby as well as the mother during the antenatal or postnatal period. Critical titer of anti-D antibody is1:8–1:16, but in anti-c antibody, it is not able to define the critical titer. After identifying the anti-c antibody in the antenatal period, it is better to plan the delivery in the hospital which has good neonatal care and blood bank facilities.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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