|Year : 2017 | Volume
| Issue : 1 | Page : 37-39
Hemolytic disease of newborn: Can think beyond Rh (D) and ABO incompatibilities
Shyam Sundar Mina, Rashmi Bhardwaj, Shobhna Gupta
Department of Paediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
|Date of Web Publication||8-Feb-2017|
Dr. Shyam Sundar Mina
Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
The widespread use of Rh-D immune globulin has led to a relative increase in the importance of non-Rh-D isoimmunization as a cause of hemolytic disease of the fetus and newborn (HDN). Disease spectrum for non-anti-D erythrocyte alloimmunization may range from subclinical hemolysis to active hemolysis and hyperbilirubinemia requiring exchange transfusion. We report such a case of HDN due to Rh anti-c in an infant of an Rh-positive mother, who required double volume exchange transfusion.
Keywords: Anti-c, hemolytic disease, hyperbilirubinemia
|How to cite this article:|
Mina SS, Bhardwaj R, Gupta S. Hemolytic disease of newborn: Can think beyond Rh (D) and ABO incompatibilities. J Clin Neonatol 2017;6:37-9
|How to cite this URL:|
Mina SS, Bhardwaj R, Gupta S. Hemolytic disease of newborn: Can think beyond Rh (D) and ABO incompatibilities. J Clin Neonatol [serial online] 2017 [cited 2019 Oct 19];6:37-9. Available from: http://www.jcnonweb.com/text.asp?2017/6/1/37/199756
| Introduction|| |
The widespread use of Rh-D immune globulin has led to a relative increase in the importance of non-Rh-D isoimmunization as a cause of hemolytic disease of the newborn (HDN). Till date, 49 Rh antigens are known. D, C, E, c, and e are among the most significant. Disease spectrum for non-anti-D erythrocyte alloimmunization depends on the type of antigen and degree of hemolysis. There is a paucity of data on the prevalence of irregular antibodies in the Indian population so that we report a case of HDN due to anti-c alloimmunization.
| Case Report|| |
A term appropriate for gestational age (2600 g) male infant born to a 26-year-old multigravida mother through vaginal route, who cried at birth, was referred to our pediatric casualty at 48 h of life with a history of yellowish discoloration noted at 36 h of life by parents. On nursery admission, the infant's general condition was fair with stable vitals. Pallor was present, and infant was deeply icteric till soles. Infant was active, alert with good spontaneous movements, normal cry, anterior fontanel at level, and normal neonatal reflexes. Respiratory and cardiovascular findings were within normal limit. Infant had hepatosplenomegaly. Risk factors for sepsis, maternal morbidity, and gross congenital anomalies were absent. There was no history of jaundice in previous siblings or jaundice in family. Serum bilirubin was found to be 40 mg/dl with indirect 36.8 mg/dl at 36 h of on life; thus, a decision of exchange transfusion was taken, and light-emitting diode phototherapy was started. Laboratory investigations demonstrated a moderate-to-severe anemia (hemoglobin 8.5 g/dl), mother's blood group A positive,reticulocyte count 14%, peripheral smear suggestive of hemolysis, and a positive direct Coombs test [Table 1].
Double volume exchange transfusion was done at 38 h of life under strict aseptic precautions. Because of the absence of apparent cause of the immune hemolytic disease such as Rh (D) or ABO incompatibilities and direct antiglobulin test (DAT) being positive, further investigation for minor blood groups was done which revealed significant alloantibody anti-c in mother's sample and a positive DAT for c antigen in the infant's sample. This established anti-c hemolytic disease due to minor blood group c incompatibility as a cause of hyperbilirubinemia in this infant. A final diagnosis of neonatal hyperbilirubinemia in a term appropriate for gestational age infant due to anti-c alloimmunization was made. Infant's jaundice reduced gradually and was discharged from nursery on the 8th day of life. Patient is in regular follow-up in high-risk clinic. Brainstem evoked response audiometry was planned at 12 weeks. Till last follow-up visit, infant growth and development were normal with no anemia.
| Discussion|| |
The Rh blood group system is the most polymorphic of the human blood groups, consisting of at least 45 independent antigens. The Rh antigens are highly immunogenic. HDN, also known as erythroblastosis fetalis, occurs when fetal red blood cells (RBCs), which possess an antigen that the mother lacks, cross the placenta into the maternal circulation, where they stimulate antibody production. The antibodies return to the fetal circulation and result in RBC destruction. It may occur due to various factors including minor blood group incompatibility also. The D antigen accounts for 50% of maternal alloimmunization. Whereas most clinically significant blood group sensitizations noted during pregnancy are still secondary to anti-D incompatibility, sensitization to antigens other than D in the CDE system is not uncommon and can cause severe hemolytic disease. The widespread use of Rh-D immunoglobulin has led to a relative increase in the non-Rh-D isoimmunization as a cause of HDN., Non-anti-D erythrocyte alloimmunization is capable of causing severe HDN include anti-c which clinically is the most important Rh antigen after the D antigen. Mild-to-moderate disease can be caused by anti-Cw and anti-Cx. Rh alloantibodies that are typically associated with mild HDN include anti-C, anti-E, and anti-e. The combination of anti-c and anti-E antigens can cause the occurrence of severe fetal and neonatal hemolytic disease.
We, therefore, hereby report such a case encountered in our neonatal unit. Currently, the frequency of subgroup incompatibilities on investigation of newborns with hyperbilirubinemia is increasing., The best-known ones among these antigens which are responsible for 3% of the cases of neonatal hemolytic disease include Kell, Duffy, Diego, Kidd, MNS, C, and E. In hemolytic disease due to subgroup incompatibility, the disease spectrum may range from subclinical hemolysis to active hemolysis and hyperbilirubinemia requiring exchange transfusion. Its diagnosis and treatment is similar to Rh (D) incompatibility. In a study performed to determine the frequency of erythrocyte alloimmunization causing hemolytic disease, the frequencies of antibodies which led to the development of hemolytic disease causing mortality among 452 neonates with a positive indirect Coombs test were as follows: anti-D - 18.4%; anti-E - 14%; anti-c - 5.8%; anti-C - 4.7%; anti-Kell - 22%; anti-MNS - 4.7%; anti-Fya (Duffy) - 5.4%; and anti-Jka - 1.5%.
The first case of HDN due to anti-c antibodies in Rh-D positive mother in India was published in a retrospective diagnosis made in 2007. The management of anti-c isoimmunization or isoimmunization with any other irregular red cell antibody is similar to the management of anti-D isoimmunized pregnancy with a specification that blood used for fetal and/or neonatal transfusion should be negative for its respective antibody., In the present case, the infant was treated with double volume exchange transfusion and phototherapy and responded well. Although non-anti-D erythrocyte alloimmunization screenings in pregnant women in terms of subgroup incompatibilities are still not recommended routinely in our country due to nonavailability, the testing for minor blood group antibodies in pregnancy and cost factor. Postnatal evaluation must be considered in any infant manifesting with severe jaundice and features of hemolysis.
| Conclusion|| |
It is thus concluded that a non-anti-D erythrocyte alloimmunization screenings in pregnant women in terms of subgroup incompatibilities are necessary for better prediction and early diagnosis of HDN. In positive cases, a close follow-up throughout pregnancy is essential for timely intervention and better outcome.
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Conflicts of interest
There are no conflicts of interest.
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