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Hyperbilirubinemia due to minor blood group (anti-E) incompatibility in a newborn: a case report.

Introduction

The frequency of neonatal hemolytic disease and indirect hyperbilirubinemias related with Rh sensitization has decreased with widespread use of anti-D gamma globulin, and the importance of minor blood group (subgroup) incompatibilities has gradually increased (1, 2).

Some patients with minor blood group incompatibility may be asymptomatic or clinical pictures ranging from active hemolysis to neonatal jaundice requiring exchange transfusion may be observed (2, 3). Minor blood group incompatibilities are responsible of 3-5% of the cases of neonatal hemolytic jaundice (4).

In this case report, a newborn who developed indirect hyperbilirubinemia due to incompatibility of E antigen, which is one of the minor blood groups, is presented because of the rarity of such cases, and minor blood group incompatibilities due to anti-E are reviewed.

Case

A male baby who was born from the third pregnancy of a 32-year-old mother with a gestational age of 32 weeks and two days and a birth weight of 3500 g by ceserean section as the third living child was discharged without any problem two days after delivery. In his prenatal history, it was learned that the mother received levothyroxine treatment in the last trimester of pregnancy because of hypothyroidism. At the follow-up visit on the fourth day after delivery, he presented with jaundice and his bilirubin level, as measured using a transcutaneous bilirubinometer was found as 18.7 mg/dL. Laboratory findings were as follows: total bilirubin: 17.6 mg/dL, direct bilirubin: 0.5 mg/dL, hemoglobin: 15.7 g/dL, white blood cells (WBC): 7730 /[mm.sup.3], platelet count: 274,000 /[mm.sup.3], blood group: 0 Rh (+), reticulocyte count: 1.56%, and direct Coombs test: (++++). Hemolysis findings or atypical cells were not observed in a peripheral smear. The mother's blood group was found as 0 Rh (+). The minor blood groups of the mother and baby tested to investigate the etiology of hyperbilirubinemia and positive direct Coombs test were as follows: mother: C(+) c(+) E(-) e(+) Kell (-), baby: C(+) c(+) E(+) e(+) Kell(-). Indirect Coombs test in the mother and anti-E antibody levels in the mother and baby were positive. Considering the clinical and laboratory findings, a diagnosis of indirect hyperbilirubinemia related with minor blood group incompatibility due to anti-E was made. The patient was treated by applying phototherapy for 28 hours without the need for advanced treatment methods such as intravenous immunoglobulin or exchange transfusion. The serum total bilirubin level was found as 10.8 mg/dL after phototherapy. On the follow-up visit one day after discharge, no "rebound" hyperbilirubinemia was found and pathologic hyperbilirubinemia did not develop in the further follow-up. Verbal informed consent was obtained from the patient's parents.

Discussion

Hemolytic disease of the newborn occurs as a result of hemolysis and shortening of the life span of the new-born's erythrocytes because of antibodies crossing from the mother by the placenta (5). Hemolysis of erythrocytes in the fetus and newborn is most frequently caused by antibodies produced due to Rh and ABO incompatibilities (2). Minor blood group incompatibilities should be considered in cases of hemolytic disease where Rh and ABO incompatibilities cannot be found and with positive Coombs tests (2, 5).

The most common minor blood group antigens that lead to blood incompatibility between the mother and baby include C, c, E, e, Kell, Duffy, Diego, Kidd, and MNSs antigen systems (3). It has been reported that the most severe hemolytic picture is caused by Anti c antibodies (5,6). Although jaundice at a level necessitating exchange transfusion did not develop in our patient, anti-E antibody was found as positive.

The pathophysiology of isoimmunization in minor blood group incompatibility in the fetus and newborn is similar to the pathophysiology of Rh incompatibility. The initial maternal antibodies produced as a response to antigenic stimulus are immunoglobulin (Ig)-M antibodies, but they have no importance in the pathogenesis of hemolytic disease of the newborn because they cannot cross the placenta. However, IgG antibodies increase with further antigenic stimuli and in antigen-positive pregnancies. These antibodies can cross the placenta and may lead to a positive indirect Coombs test in the mother. Thus, they cause hemolytic disease in the fetus and newborn with varying severity (2, 5). In the case presented in this article, the direct

Coombs test, indirect Coombs test in the mother, and anti-E antibody levels were positive and the diagnosis of hyperbilirubinemia caused by minor blood group incompatibility due to anti-E was confirmed.

In cases of hemolytic disease due to minor blood group incompatibility, the picture may vary from subclinical hemolysis to active hemolysis and hyperbilirubinemia, which require exchange transfusion (2, 3, 5). Early and severe pictures such as hydrops fetalis (7) or later and mild pictures such as prolonged jaundice (5) may also be observed.

Variability of clinical findings related with minor blood group incompatibility is also valid for blood group E. To et al. (8) reported that serious hyperbilirubinemia did not develop in a newborn whose mother had a positive indirect Coombs test in the screening performed in the 15th week of pregnancy and who had positive anti-E after delivery. On the other hand, Sarici et al. (1) reported a newborn who was diagnosed as having hemolytic disease due to anti-E and had severe hyperbilirubinemia that necessitated exchange transfusion twice on the postnatal fourth day (total bilirubin level 36 mg/dL). Onesimo et al. (9) reported that they obtained successful results with phototherapy and intravenous (IV)-IG in E subgroup incompatibility. Our patient was also successfully treated with phototherapy without the need for IVIG or exchange transfusion. This clinical prognosis might have arisen from the relatively mild anti-E hemolytic disease prognosis and/or close and strict follow-up after discharge, which is practiced in our unit. In this way, diagnosis could be made and treatment was administered before the hyperbilirubinemia reached significant levels.

Another finding in our case that directed us to investigations that would confirm the diagnosis was the direct Coombs positivity found during the investigation of the cause of hyperbilirubinemia. In minor blood group incompatibilities, the rate of direct Coombs test positivity is generally 33% (2, 5, 10). It should be kept in mind that a direct Coombs test may be positive (3) or negative (2, 7) in E blood group incompatibilities.

In conclusion, physicians should consider that minor blood group incompatibilities may be involved in the etiology of newborns who present with jaundice and have a positive direct Coombs test.

Informed Consent: Verbal informed consent was obtained from patients' parents who participated in this case.

Peer-review: Externally peer-reviewed.

Author Contributions: Concept - S.U.S.; Design - S.S.; Supervision - S.U.S.; Resources - Y.Y., M.O.; Materials - M.O.; Data Collection and/or Processing - M.O.; Analysis and/or Interpretation - V.B.E.; Literature Search - Y.Y., M.O.; Writing Manuscript - S.U.S., M.O.; Critical Review - S.U.S.

Conflict of Interest: No conflict of interest was declared by the authors.

Financial Disclosure: The authors declared that this study has received no financial support.

References

(1.) Sarici SU, Alpay F, Yesilkaya E, Ozcan O, Gokcay E. Hemolytic disease of the newborn due to isoimmunization with anti-E antibodies: a case report. Turk J Pediatr 2002; 44: 248-50.

(2.) Gokce IK, Guzoglu N, Oncel MY, Calisici E, Canpolat FE, Dilmen U. Yenidogan doneminde anemi ile semptom veren minor kan grubu (anti-C ve anti-E) uygunsuzluguna bagli hemolitik hastalik. Turk J Pediatr Dis 2014; 1: 32-4. [CrossRef]

(3.) Ozdemir OMA, Kucuktasci K, Sahin O, Eliacik C, Ergin H. Yenidoganda anti-E'ye bagli subgrup uyusmazligi: iki olgu sunumu. ADU Tip Fak Derg 2014; 15: 77-8.

(4.) Ozkaya H, Bahar A, Ozkan A, Karademir F, Gocmen I, Mete Z. Indirekt hiperbilirubinemili yenidoganlarda ABO, Rh ve subgrup (Kell, c,e) uyusmazliklari. Turk Pediatri Ars 2000; 35: 30-5.

(5.) Can E, Ozkaya H, Meral C, et al. Anti-C'ye bagli yenidoganin hemolitik hastaligi ve uzamis sariligi: Uc vaka takdimi. Cocuk Sagligi ve Hastaliklari Dergisi 2009; 52: 88-90.

(6.) Liley HG. Immune hemolytic disease of the newborn. In: Nathan DG, Oski FA, (eds). Hematology of infancy and childhood. 7th ed. Vol 1. Philadelphia: WB Saunders; 2009.p. 89-92.

(7.) Bas EK, Bulbul A, Uslu S, Arslan S, Celik M, Nuhoglu A. Nadir bir durum: anti E'ye bagli subgrup uyusmazligi. Turk Pediatri Ars 2013; 48: 80-1.

(8.) To WW, Ho SN, Mok KM. Anti-E alloimmunization in pregnancy: Management dilemmas. J Obstet Gynaecol Res 2003; 29: 45-8. [CrossRef]

(9.) Onesimo R, Rizzo D, Ruggiero A, Valentini P. Intravenous immunoglobulin therapy for anti-E hemolytic disease in the newborn. J Matern Fetal Neonatal Med 2010; 23: 1059-61. [CrossRef]

(10.) Zipursky A, Bowman JM. Isoimmune hemolytic diseases. In: Nathan DG, Oski FA, (eds). Hematology of infancy and childhood. 6th ed. Vol 1. Philadelphia: WB Saunders; 2003: 44-73.

Murat Ozcan (1), Selin Sevinc (1), Vildan Boz Erkan (1), Yuksel Yurdugul (1), S. Umit Sanci (2)

(1) Department of Pediatrics, Ufuk University Faculty of Medicine, Ankara, Turkey

(2) Department of Pediatrics, Division of Neonatology, Ufuk University Faculty of Medicine, Ankara, Turkey

Address for Correspondence: Murat Ozcan

E-mail: dr.muratozcan@hotmail.com

Received: 09.02.2015

Accepted: 12.06.2015

DOI: 10.5152/TurkPediatriArs.2017.2658

Cite this article as: Ozcan M, Sevinc S, Boz Erkan V, Yurdugul Y, Sarici SU. Hyperbilirubinemia due to minor blood group (anti-E) incompatibility in a newborn: a case report. Turk Pediatri Ars 2017; 52: 162-4.
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Title Annotation:Case Report
Author:Ozcan, Murat; Sevinc, Selin; Erkan, Vildan Boz; Yurdugul, Yuksel; Sanci, S. Umit
Publication:Turkish Pediatrics Archive
Article Type:Case study
Date:Sep 1, 2017
Words:1549
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