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1 대한수혈학회지 : 제 27 권제 2 호, 2016 The Korean Journal of Blood Transfusion Vol. 27, No. 2, , August pissn eissn Case Report 항 -S 항체에의한태아신생아용혈성질환 1 예 : 국내첫보고 육희정 1, * ㆍ김영곤 1, * ㆍ정유나 1 ㆍ곽정숙 1 ㆍ김하늬 1 ㆍ이은희 2 ㆍ김대원 1 고려대학교의과대학진단검사의학교실 1, 소아과학교실 2 A Case of Hemolytic Disease of the Fetus and Newborn due to Anti-S Antibody: The First Case in Korea Hee-Jeong Youk 1, *, Young-gon Kim 1, *, Yoo Na Chung 1, Jung suk Kwag 1, Ha-Nui Kim 1, Eun Hee Lee 2, Dae-Won Kim 1 Departments of Laboratory Medicine 1 and Pediatrics 2, College of Medicine, Korea University, Seoul, Korea A full term male infant was admitted to the neonatal intensive care unit due to jaundice and mild hemolytic anemia within the first 24 hours of his life. The total serum bilirubin level was 11.2 mg/dl at 24 hours of age. The patient was RhD positive and blood group A, and his mother was RhD positive and blood group B. The direct and indirect antiglobulin tests of the infant were all positive. On antibody screening and identification tests, anti-s antibodies were identified from both the infant and mother. The RBC phenotyping for S antigen revealed positive for infant and negative for mother. This report documents the first case of hemolytic disease of the fetus and newborn due to the anti-s antibody in Korea. (Korean J Blood Transfus 2016;27: ) Key words: Anti-S antibody, Hemolytic disease, Fetus and newborn 서론태아신생아용혈성질환은산모에게결여된항원을가진태아의적혈구가모체의순환계로들어가모체에서이항원에대한항체가생성되고, 모체의 IgG 항체가태반을통과해태아의순환으로유입되면태아적혈구표면항원에부착되어용혈이일어나는질환으로빈혈과황달이주된임 상소견이다. 1,2) 태아신생아용혈성질환은용혈반응이없고단지태아적혈구의 IgG 감작만일으키는경우에서부터핵황달 (Kernicterus) 과같은생명의위험을가져올수있는경우까지다양한임상양상의차이를보인다. 1,3) 따라서태아신생아용혈성질환은조기에진단하여예방및신속한치료를해야한다. 태아신생아용혈성질환은 ABO 부적합또는비 Received on July 22, Revised on August 5, Accepted on August 9, 2016 Correspondence to: Dae-Won Kim Department of Laboratory Medicine, Korea University Anam Hospital, 73 Inchon-ro, Seongbuk-gu, Seoul 02841, Korea Tel: , Fax: , dwkim83493@kumc.or.kr *Hee-Jeong Youk and Young-gon Kim contributed equally as co-first authors. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Copyright C 2016 The Korean Society of Blood Transfusion

2 Korean J Blood Transfus Vol. 27, No. 2, , Aug 예기항체에의하여발생한다. 최근 Rh 면역글로불린사용으로항-D에의한태아신생아용혈성질환의빈도는감소하였고다른비예기항체에의한태아신생아용혈성질환의사례가상대적으로증가하고있다. 1,2) 1941년 Levine 등 4) 이비정형응집소에의한태아신생아용혈성질환을처음보고하였고, 국내에서는 1981년 Kim 등 5) 에의해항-E 항체에의한태아신생아용혈성질환이보고된이래, 여러비예기항체에의한태아신생아용혈성질환에대한증례보고가있었다. MNS 혈액형군에속하는 S 혈액형항원에대한항-S 항체에의한태아신생아용혈성질환의보고는국외에서몇몇사례가보고되었는데, 문헌에의하면경도의용혈성질환에서부터중등도의용혈성질환을일으키는것으로알려져있다. 3,6-11) 국내에서본증례와같이항-S 항체에의한태아신생아용혈성질환에대한보고는찾을수없었다. 2,12) 이에저자들은경도의태아신생아용혈성질환을보인환아와환아의모체혈청에서태아신생아용혈성질환의원인인항-S 항체를국내에서는처음으로동정하였기에보고하는바이다. Table 1. Results of agglutination reactions of immunohematologic tests in the infant and mother Infant Mother Direct antiglobulin test Polyspecific anti-igg/c3d 3+ 0 Monospecific anti-igg 3+ 0 Monospecific anti-c3d 0 0 Reactivity of RBCs with Anti-S sera , several large agglutinates; 1+, turbid background with small agglutinates; 0, no agglutination. Abbreviation: RBCs, red blood cells. 증례과거수혈받은적이없으나 2년전자궁외임신의과거력이있는 35세산모가체중 3.34 kg인남아를재태기간 39주 4일에자연분만으로출산하였다. 환아의출생당시의상태는양호하였으나생후약 10시간이후몸전체에서황달소견을보였다. 환아는총빌리루빈증가와함께직접항글로불린및간접항글로불린검사상양성소견으로태아신생아용혈성질환의심하에신생아집중치료실로전실되었다. 입원당시시행한일반혈액검사상혈색소치는 12.2 g/dl ( 참고범위 g/dl), 백혈구수 15,500 / L (6,000 21,000 / L), 혈소판수는 142,000 / L (150, ,000 / L) 이었으며, 망상적혈구는 7.6% ( % of RBC) 였다. 말초혈액도말검사상유핵적혈구가백혈구 100개당 11개보였고, 적혈구형태는중등도의적혈구부동증 (anisocytosis) 과변형적혈구증 (poikilocytosis) 을보였다. 출생후 24시간총빌리루빈치는 11.2 mg/dl ( mg/dl), haptoglobin치는 <5 mg/dl ( mg/dl), lactate dehydrogenase는 1,541 IU/L ( IU/L), aspartate aminotransaminase은 82 IU/L (3 45 IU/L) 이었다. 산모의혈액형은 B형, RhD 양성이었고, 환아는 A형, RhD 양성이었다. 원주응집법 (column agglutination technique) 으로시행한직접항글로불린검사에서산모는음성, 환아는 LISS/Coombs gel card (Biorad, Cressier, Switzerland) 의다특이성항글로불린시약 (polyspecific antihuman globulin) 과 DC Screening II (Biorad, Cressier, Switzerland) 의 monospecific anti-igg를사용한검사에서 3+였고, monospecific anti-c3d를사용한검사에서는음성결과였다 (Table 1). Anti-human globulin (AHG) polyspecific cassette

3 육희정외 : 항 -S 항체에의한태아신생아용혈성질환 1 예 : 국내첫보고 Table 2. Results of antibody screening and identification test on the infant and mother Rh-hr Kell Duffy Kidd Xg Lewis MNS P Luth. Infant Mother Cell# Enzyme AHG polyspecific Enzyme D C E c e f C w V K k Kp a Kp b Js a Js b Fy a Fy b Jk a JK b Xg a Le a Le b S s M N P1 Lu a Lu b AHG polyspecific I / II / / / s / / / / / / Auto Abbreviations: /, not tested; AHG, anti-human globulin. (Ortho Clinical Diagnostics, Raritan, USA) 와 0.8% Selectogen I, II 를사용하여 AutoVue Innova (Ortho Clinical Diagnostics, Raritan, USA) 를이용한비예 기항체선별검사에서환아와산모모두양성반 응을보였으며, 비예기항체동정검사로 AHG polyspecific cassette 를이용한 3% Resolve Panel A (Ortho Clinical Diagnostics, Raritan, USA) 에서환 아와산모모두동일한항 -S 항체가동정되었다. 추가검사로환아와산모모두에게시행한 Neutral cassette 의 3% Resolve Panel C Ficin Treated (Ortho Clinical Diagnostics, Raritan, USA) 에서모든동정 혈구에는음성결과를나타내었다 (Table 2). 항 -S 항혈청 (Diagast, Loos, France) 을이용하여산모와 환아적혈구의 S 항원표현형검사를시행한결 과, 환아는양성, 산모는음성이었다 (Table 1). 환아는생후 24 시간이내에총빌리루빈치가 고위험군에속하였지만 25 mg/dl 를넘지않았으 므로교환수혈은시행하지않았고광선치료만시행하였고, 이후 6일간광선치료를유지하였다. 환아는생후 7일째에혈색소치 11.4 g/dl, 총빌리루빈치는 9.40 mg/dl로저위험군에속하였고전신상태가양호하여생후 8일째퇴원하였다. 외래추적검사시총빌리루빈은생후 14일째 3.62 mg/dl 로감소하였다. 고찰 항-S항체는 MNSs 혈액형군에속하는항원들중 S항원에대한항체이다. 1947년 Sanger와 Race 13) 가 S항원이유전학적으로 MN계와관련이있음을보고하였고, 1951년 Levine 등 14) 이 S항원에의한태아신생아용혈성질환을처음으로보고하였다. MN 항원에대한항체는대개자연발생적으로생기며체온에서반응하지않는한임상적인문제를일으키는것은드물다고알려져있지만, S

4 Korean J Blood Transfus Vol. 27, No. 2, , Aug 에대한항체들은용혈성수혈반응과태아신생아용혈성질환을일으키는것으로알려져있다. 1,6-11) 한국인의 S 항원양성율은 9% 로알려져있으며 1) 국내에서항-S 항체의동정이 7년간의단일기관분석으로성인에서 6건이보고된바있으나항-S 항체에의한태아신생아용혈성질환은보고된바가없다. 2,12) 이에대한설명으로는산모에서발견되는동종항체중약 2% 의낮은빈도로검출되는비예기항체이기때문인것으로여겨진다. 11,15) 환아는출생후 24시간이되기이전에경도의빈혈과황달을보이는등전형적인태아신생아용혈성질환의임상상을보였으며, 산모와환아의혈액형검사상 ABO와 RhD에의한태아신생아용혈성질환을배제할수있었다. 산모와환아에서시행한항체동정검사에서동일하게항-S 항체가동정되었고, 항원표현형검사에서산모는 S항원음성, 환아는 S항원양성이확인되어비예기항체동정검사에서동정된항-S 항체에의한태아신생아용혈성질환으로진단할수있었다. 환아는산모의첫번째아기지만, 산모는출산 2년전자궁외임신의과거력이있었고수혈의과거력은없었으므로, 당시의자궁외임신으로인해아기아버지의 S항원에대해감작되었음을배제할수없다. 본증례에서는환아아버지에대해 S항원검사는시행하지못했다. 대부분의국외사례들과마찬가지로본증례는교환수혈없이광선치료만시행했던경도의태아신생아용혈성질환의사례로, 항-S 항체에의한태아신생아용혈성질환의첫번째국내증례로보고하는바이다. 요약 만삭남아가출생첫 24시간이내보인황달과경도의용혈성빈혈로신생아집중치료실에입원하 였다. 총빌리루빈수치는출생첫 24시간에 11.2 mg/dl이었다. 환아의혈액형은 A형 RhD 양성이었고, 산모는 B형 RhD 양성이었다. 환아의직접및간접항글로불린검사는양성이었고항체선별검사및항체동정검사결과환아와산모모두항-S 항체가동정되었고산모와환아에게시행한 S항원에대한적혈구표현형검사결과산모는음성, 환아는양성을보였다. 본증례는국내에서항-S 항체에의한태아신생아용혈성질환의첫보고이다. References 1. Han KS, Park KU, Song EY. Transfusion medicine. 4th ed. Seoul: Korea Medical Book Publisher, 2014:100-6, Song EY, Han BY, Hwang DH, Choi JH, Park SS, Kim EC, et al. Analysis of antibodies causing hemolytic disease of the newborn. Korean J Blood Transfus 1998;9: Weinstein L. Irregular antibodies causing hemolytic disease of the newborn: a continuing problem. Clin Obstet Gynecol 1982;25: Levine P, Katzin EM, Burnham L. Soimmunization in pregnancy its possible bearing on the etiology of erythroblastosis foetalis. JAMA 1941;116: Kim WB, Yoo KS, Lee DW, Kang DY, Shin SM, Lee SJ. A case of hemolytic disease of newborn due to anti-e. Korean J Hematol 1981;16: Reid ME. MNS blood group system: a review. Immunohematology 2009;25: Griffith TK. The irregular antibodies--a continuing problem. Am J Obstet Gynecol 1980; 137: Pitan C, Syed A, Murphy W, Akinlabi O, Finan A. Anti-S antibodies: an unusual cause of haemolytic disease of the fetus and newborn

5 육희정외 : 항 -S 항체에의한태아신생아용혈성질환 1 예 : 국내첫보고 (HDFN). BMJ Case Rep 2013;2013:bcr Levine P, Ferraro LR, Koch E. Hemolytic disease of the newborn due to anti-s; a case report with a review of 12 anti-s sera cited in the literature. Blood 1952;7: Mayne KM, Bowell PJ, Green SJ, Entwistle CC. The significance of anti-s sensitization in pregnancy. Clin Lab Haematol 1990;12: Reddy VS, Kohan R. Severe hemolytic disease of fetus and newborn due to anti-s antibodies. J Clin Neonatol 2014;3: Lee HJ, Jo SY, Shin KH, Song D, Lee SM, Kim IS, et al. Analysis of unexpected antibodies detected in children: a single center study for 7 years. Korean J Blood Transfus 2015;26: Sanger R, Race RR. Subdivisions of the MN blood groups in man. Nature 1947;160: Levine P, Kuhmichel AB, Wigod M, Koch E. A new blood factor, s, allelic to S. Proc Soc Exp Biol Med 1951;78: Dajak S, Stefanović V, Capkun V. Severe hemolytic disease of fetus and newborn caused by red blood cell antibodies undetected at first-trimester screening (CME). Transfusion 2011;51:

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untitled 대한수혈학회지 : 제 19 권제 1 호, 2008 항 -E, 항 -M, 항 -Jk b, 항 -Le a 복합항체가검출된환자에서의용혈성수혈부작용경험 1 예 이종한ㆍ이상국ㆍ배인철ㆍ백은정ㆍ김신영ㆍ김현옥 = Abstract = 연세대학교의과대학진단검사의학교실 A Case of Hemolytic Transfusion Reaction in a Patient with Anti-E,