In addition to Rh and ABO incompatibilities subgroup incompatibilities may rarely play a role among the causes of hemolytic anemia and indirect hyperbilirubinemia in newborns. The most common minor blood group antigens that cause blood incompatibility between the mother and baby are C, c, E, e, Kell, Duffy, Diego, Kidd and MNSs antigens. In this article, a newborn in whom hyperbilirubinemia due to anti-E minor blood group incompatibility developed and was treated with phototherapy succesfully is presented and minor blood group incompatibilities due to anti-E are reviewed

Erkan, Vildan Boz

Sarıcı, Serdar Ümit

Sevinç, Selin

Yurdugül, Yüksel

Özcan, Murat

English

Ufuk Universitesi Akademik Acikerisim Sistemi

Case ReportAbstractIn addition to Rh and ABO incompatibilities subgroup incompatibilities may rarely play a role among the causes of hemolytic anemia and in-direct hyperbilirubinemia in newborns. The most common minor blood group antigens that cause blood incompatibility between the mother and baby are C, c, E, e, Kell, Duffy, Diego, Kidd and MNSs antigens. In this article, a newborn in whom hyperbilirubinemia due to anti-E minor blood group incompatibility developed and was treated with phototherapy succesfully is presented and minor blood group incompatibilities due to anti-E are reviewed.Key words: Anti-E, hyperbilirubinemia, minor blood group incompatibility, newborn 162Hyperbilirubinemia due to minor blood group (anti-E) incompatibility in a newborn: a case reportMurat Özcan1, Selin Sevinç1, Vildan Boz Erkan1, Yüksel Yurdugül1, S. Ümit Sarıcı2¹ Department of Pediatrics, Ufuk University Faculty of Medicine, Ankara, Turkey² Department of Pediatrics, Division of Neonatology, Ufuk University Faculty of Medicine, Ankara, TurkeyAddress for Correspondence: Murat Özcan   E-mail: dr.muratozcan@hotmail.com  Received: 09.02.2015 Accepted: 12.06.2015©Copyright 2017 by Turkish Pediatric Association - Available online at www.turkpediatriarsivi.comDOI: 10.5152/TurkPediatriArs.2017.2658IntroductionThe frequency of neonatal hemolytic disease and in-direct hyperbilirubinemias related with Rh sensiti-zation has decreased with widespread use of anti-D gamma globulin, and the importance of minor blood group (subgroup) incompatibilities has gradually in-creased (1, 2).Some patients with minor blood group incompatibility may be asymptomatic or clinical pictures ranging from active hemolysis to neonatal jaundice requiring ex-change 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 indi-rect 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 mi-nor blood group incompatibilities due to anti-E are reviewed.CaseA 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 his-tory, it was learned that the mother received levothy-roxine treatment in the last trimester of pregnancy be-cause 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 transcuta-neous bilirubinometer was found as 18.7 mg/dL. Lab-oratory 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 /mm³, platelet count: 274,000 /mm³, blood group: 0 Rh (+), reticulocyte count: 1.56%, and direct Coombs test: (++++). Hemolysis find-ings 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 Cite this article as: Özcan M, Sevinç S, Boz Erkan V, Yurdugül Y, Sarıcı SÜ. Hyperbilirubinemia due to minor blood group (anti-E) incompatibility in a newborn: a case report. Turk Pediatri Ars 2017; 52: 162-4.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. Consider-ing the clinical and laboratory findings, a diagnosis of indirect hyperbilirubinemia related with minor blood group incompatibility due to anti-E was made. The pa-tient was treated by applying phototherapy for 28 hours without the need for advanced treatment methods such as intravenous immunoglobulin or exchange transfu-sion. 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 con-sent 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 eryth-rocytes in the fetus and newborn is most frequently caused by antibodies produced due to Rh and ABO in-compatibilities (2). Minor blood group incompatibili-ties 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 anti-bodies (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 new-born is similar to the pathophysiology of Rh incom-patibility. The initial maternal antibodies produced as a response to antigenic stimulus are immunoglobu-lin (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 severi-ty (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 in-compatibility 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 indi-rect Coombs test in the screening performed in the 15th week of pregnancy and who had positive anti-E after de-livery. On the other hand, Sarıcı et al. (1) reported a new-born who was diagnosed as having hemolytic disease due to anti-E and had severe hyperbilirubinemia that necessitated exchange transfusion twice on the postna-tal 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 sub-group incompatibility. Our patient was also successful-ly 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, di-agnosis could be made and treatment was administered before the hyperbilirubinemia reached significant levels.Another finding in our case that directed us to investi-gations 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 positiv-ity 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. 163Turk Pediatri Ars 2017; 52: 162-4 Özcan et al. Minor blood group incompatibility in a newbornAuthor Contributions: Concept - S.Ü.S.; Design - S.S.; Super-vision - S.Ü.S.; Resources - Y.Y., M.Ö.; Materials - M.Ö.; Data Collection and/or Processing - M.Ö.; Analysis and/or Interp-retation - V.B.E.; Literature Search - Y.Y., M.Ö.; Writing Ma-nuscript - S.Ü.S., M.Ö.; Critical Review - S.Ü.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.References1. Sarici SU, Alpay F, Yeşilkaya E, Ozcan O, Gökçay E. He-molytic disease of the newborn due to isoimmunization with anti-E antibodies: a case report. Turk J Pediatr 2002; 44: 248-50.2. Gökçe İK, Güzoğlu N, Öncel MY, Çalişici E, Canpolat FE, Dilmen U. Yenidoğan döneminde anemi ile semptom veren minör kan grubu (anti-C ve anti-E) uygunsuzluğu-na bağlı hemolitik hastalık. Turk J Pediatr Dis 2014; 1: 32-4. [CrossRef ]3. Özdemir ÖMA, Küçüktaşçı K, Şahin Ö, Eliaçık Ç, Ergin H. Yenidoğanda anti-E’ye bağlı subgrup uyuşmazlığı: iki olgu sunumu. ADU Tip Fak Derg 2014; 15: 77-8.4. Özkaya H, Bahar A, Özkan A, Karademir F, Göçmen İ, Mete Z. İndirekt hiperbilirubinemili yenidoğanlarda ABO, Rh ve subgrup (Kell, c,e) uyuşmazlıkları. Turk Pedi-atri Ars 2000; 35: 30-5.5. Can E, Özkaya H, Meral C, et al. Anti-C’ye bağlı yeni-doğanın hemolitik hastalığı ve uzamış sarılığı: Üç vaka takdimi. Çocuk Sağlığı ve Hastalıkları 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. Baş EK, Bülbül A, Uslu S, Arslan S, Çelik M, Nuhoğlu A. Nadir bir durum: anti E’ye bağlı subgrup uyuşmazlığı. 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. Intraveno-us 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: 44-73.164Turk Pediatri Ars 2017; 52: 162-4Özcan et al. Minor blood group incompatibility in a newborn

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

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

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