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1 대한수혈학회지 : 제 27 권제 1 호, 2016 The Korean Journal of Blood Transfusion Vol. 27, No. 1, 79-83, April pissn eissn Case Report 효소반응단계에서소실되는항 -Jk b 에의한용혈수혈반응 1 예 송새암ㆍ오승환ㆍ이자영ㆍ성성경ㆍ신정환ㆍ김혜란ㆍ전경란ㆍ이정녀 인제대학교의과대학진단검사의학과 A Case of Hemolytic Transfusion Reaction Attributable to Anti-Jk b Abolished in the Enzyme Phase Reaction Sae Am Song, Seung Hwan Oh, Ja Young Lee, Sung Kyung Sung, Jeong Hwan Shin, Hye Ran Kim, Kyung Ran Jun, Jeong Nyeo Lee Department of Laboratory Medicine, Inje University College of Medicine, Busan, Korea We report a case of an intravascular hemolytic reaction attributable to anti-jk b antibodies that were not detected using an enzyme phase antibody identification test. A 61-year-old male who had received two units of red blood cells was admitted to the emergency room because his urine was dark. LISS/Coombs gel column agglutination tests suggested the presence of anti-jk b and anti-e antibodies. However, his serum was negative for the Jk b antigen when an enzyme phase test was performed. A positive reaction was evident, however, when EDTA-treated plasma was tested; this excluded any possible complement-mediated reaction. The patient was diagnosed with an intravascular hemolytic transfusion reaction, caused by anti-jk b, and was later discharged without specific complications after receiving antigen-negative blood transfusions. (Korean J Blood Transfus 2016;27:79-83) Key words: Kidd Blood-Group System, Enzyme, EDTA, Hemolytic transfusion reaction 서론 Kidd 혈액형군은 Jk a, Jk b 의두가지항원으로주로구성되어있으며이는적혈구막을통한요소수송에중요한역할을하는것으로알려져있다. 1) Jk a 항원은 1951년신생아용혈성질환을보이는신생아를출산한산모의혈청으로부터규명된이후, 2) 1953년항-Jk b 에의한동종면역반응의 첫사례가보고되었다. 3) Jk a 와 Jk b 항원의발현빈도는백인에서각각 77%, 72% 로높게나타나며 4) 한국인에서의발현빈도도이와유사하게약 67%, 77% 로나타났다. 5) 항-Jk는대부분 IgG 면역항체로 50% 에서보체와결합하며태반통과가가능하여신생아용혈성질환을유발할수있으며 4,6) 또한지연성용혈성수혈부작용을일으키는원인항체의 1/3 이상을 Received on December 16, Revised on January 15, Accepted on January 21, 2016 Correspondence to: Seung Hwan Oh Department of Laboratory Medicine, Inje University College of Medicine, 75 Bokji-ro, Busanjin-gu, Busan 47392, Korea Tel: , Fax: , paracelsus@daum.net 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. 1, 78-83, Apr 차지한다고알려져있다. 하지만항-Jk는항원의자극이없어지면항체역가도급감하였다가항원노출시급증하는특징을가지므로수혈전검사에서는검출이되지않거나약한반응을보였다가수혈시심한용혈수혈반응을일으킬수있어검출과판독에주의가필요하다. 대개항-Jk는항글로불린법으로검출되며 polyethylene glycol (PEG) 이나효소처리적혈구사용시반응강도가증강되므로항체의정확한동정을위해서는효소법을함께시행하는것이도움이되는것으로알려져있다. 7) 하지만일부증례에서는 LISS/Coombs법에서항-Jk가의심되어효소법으로항체동정검사를시행하였을때오히려항체반응이소실되는사례가보고되었으며이는모두항-Jk a 였다. 8,9) 본증례에서는효소법에서항체반응이소실된항- Jk b 의사례와이에의한혈관내용혈수혈반응 1예를경험하였기에이를보고하고자한다. 증례 61세남자환자로내원 2일전발생한적갈색소변을주소로응급실에내원하였다. 환자는특이병력없었던분으로내원 9일전흑색변을본후중소병원에서 1단위농축적혈구수혈을받았고이틀후추가적으로 1단위농축적혈구수혈을받았으며당시특별한이상징후는없었다. 두번째수혈을받은 5일후적갈색소변이시작되었으며직전에구토, 구역, 어지럼증이함께동반되어개인병원내원후복부 CT검사를시행하였으나이상소견은발견되지않았다. 그러나혈액검사상 AST, LDH 등의상승소견을보여용혈성빈혈과헤모글로빈뇨증의심하에전원되었다. 당시시행한혈액검사에서혈색소 8.7 g/dl, 혈소판수 226,000/ L, 백혈구수 5,490/ L였으며직접및간접항글로불린검사에서모두양성소견을보였 다. 총빌리루빈 2.9 mg/dl ( 참고범위 mg/dl), LDH 1254 U/L ( U/L), AST 186 U/L (10 33 U/L) 로증가되어있었으며 haptoglobin <20 mg/dl ( mg/dl) 로감소되어있었다. 소변검사에서는 blood 3+, 적혈구 0 2/HPF, 소변 hemosiderin 음성, hemoglobin 양성소견을보여혈관내용혈수혈반응이의심되었다. LISS/Coombs card법을이용한비예기항체선별검사 (ID-Diacell I-II Lot No x, Bio-Rad, Cressier-sur-Morat, Switzerland) 에서양성반응을보였으며항체동정검사 (ID-DiaPanel & DiaPanel-P Lot No x & x, Bio-Rad, Cressier- sur-morat, Switzerland) 에서항-E 항체와항-Jk b 가의심되었다. 추가로혈청을 (clot activator and silicone coated, REF , Becton, Dickison and Co. (BD), Franklin Lakes, NJ, USA) 사용하여효소법을시행한결과 E 항원양성패널적혈구와의반응강도는증강되었으나항-Jk b 의경우반응이오히려소실되었으며동정혈구의제조번호 (ID-DiaCell I-II Lot No x, ID-DiaPanel, DiaPanel-P Lot No x & x, Bio-Rad, Cressier-sur-Morat, Switzerland) 를달리하여재검을실시하였으나동일한결과를보였다. 이에보체결합반응을억제하기위해일반적으로비예기항체동정검사에사용하고있던혈청검체대신 ethylenediaminetetraacetic acid 용기 (K2 EDTA 5.4 mg REF , BD) 에검체를채취한후혈장을이용하여검사를재시행한결과효소법에서항체반응이나타나항-Jk b 를확인할수있었다 (Table 1). 환자는내원 2일째부터정상소변색을보였으며내원 3일에는 E 항원과 Kidd 항원음성농축적혈구 2단위를수혈하였으며특이적인합병증없이퇴원하였다

3 송새암외 : 효소반응단계에서소실된항 -Jk b 1 예 Table 1. RBC panels and results of unexpected antibody screening test and identification test (ID-DiaCell I-II Lot No x, ID-DiaPanel and DiaPanel-P Lot No x and x) Cell D C E c e C w K k Kp a Kp b Js a Js b Fy a Fy b Jk a Jk b Le a Le b P1 M N S s Lu a Lu b Xg LISS/ Coombs LISS/Coombs Enzyme (EDTA*) Enzyme (EDTA*) I nt nt nt 2+ nt II nt nt nt 2+ nt nt nt / nt nt nt nt nt nt nt nt nt nt nt nt nt nt nt nt nt nt nt Auto *EDTA-plasma was used to inhibit antigen-antibody reaction by complement. Abbreviation: nt, not tested

4 Korean J Blood Transfus Vol. 27, No. 1, 78-83, Apr 고찰 항 -Jk b 에의한지연성용혈성수혈부작용은 1957년첫보고된이후 10) 신생아용혈성질환을비롯한여러수혈부작용등이국내외에서간헐적으로보고되어져왔다. 4,6,11,12) 일반적으로 Kidd군의적혈구항체는혈관외용혈을일으키며증상이경미하여치명적인경우는드문것으로알려져있으나일부에서는혈관내용혈로신부전등과같은심각한부작용을야기하기도한다. 11,12) 항-Jk b 는일반적인비예기항체검사에서검출되지않을수있으며이는항체의면역원성이낮고항원에재노출되기전까지는역가가낮기때문인것으로생각된다. 본증례에서도첫번째수혈시에는특이적인반응을보이지않다가두번째수혈후 5일째혈관내용혈의심증상이나타났으며이는이전에형성된항체의기억반응에의한용혈수혈반응으로인한것으로판단된다. 본증례에서환자의수혈전항체검출여부는확인되지않았으나환자는수혈후용혈수혈반응의전형적인임상양상을보였으며비예기항체검사에서항-Jk b 가동정되었다. 이후수혈시에는 Jk b 항원음성혈액을선별하여불출하였으며환자는수액요법만으로증상이호전되었다. 그러나항-Jk b 의동정과정에있어일반적으로알려진것과는달리효소법을이용한비예기항체동정검사에서오히려반응이소실되어판독에혼란을야기하였다. 몇몇국내논문에의하면효소법에서항체반응이소실된항-Jk a 의증례가보고되었으나본증례와같이항-Jk b 의경우는보고된바가없었다. 항-Jk a 나항-Kell의경우보체결합성동종항체로효소처리적혈구와의반응에서보체에의해항원-항체응집반응이억제될수있다고알려져있으며, 13,14) 보체불활성화물질인 EDTA를첨가하면보체에의한억제반응이소실될수있다 고하였다. 따라서 LISS/Coombs법에서반응이관찰되나효소법에서소실된경우, 본증례와같이 EDTA 검체가이용가능하다면 EDTA 혈장을사용하여동정을시도하고, 그렇지않은경우동정용적혈구 50 L와혈청 25 L에 0.2 M EDTA를동량으로혼합한후 37 o C에서 30분간반응시키는방법 8) 을고려해볼수있다. 본증례에서도 EDTA 혈장을이용하여재검사를시행한결과효소법에서소실되었던항원-항체응집반응이나타나는것을확인할수있었다. 비예기항체선별및동정검사는안전한수혈을위한기본적인검사로국내대부분의검사실에서시행되고있으며여러논문에서항체동정률향상에있어효소법의유용성이보고되면서 15) LISS/Coombs법과함께효소법을시행하는기관도증가하였다. 하지만본증례와같이효소법에서전형적인항체반응양상이나타나지않을경우오히려판독시에혼란을발생시킬수있으므로, 비전형적인반응양상을인지하고여러가지방법을이용하여항체동정을적극적으로시도하여야할것으로생각된다. 특히항-Jk b 는용혈성수혈부작용을일으키는원인이될수있으므로반드시항원음성혈액을수혈하여야하므로효소법에서는사용된검체에따라비전형적반응을보일수있음을인지하고이를해결하는방법을숙지하여야하겠다. 요약 저자들은효소법에서항체반응이소실된항-Jk b 의사례와이에의한혈관내용혈성수혈부작용 1례를경험하였기에이를보고하고자한다. 환자는 61세남자환자로 2단위적혈구수혈후적갈색소변을주소로내원하였다. LISS/Coombs card 법을이용한비예기항체검사에서항-E와항-Jk b

5 송새암외 : 효소반응단계에서소실된항 -Jk b 1 예 가의심되었으나, 효소법에서 Jk b 항원적혈구와의항체반응은음성이었다. 이에보체결합에의한억제반응으로생각되어 EDTA 혈장을이용하여반응시킨결과항-Jk b 에의한용혈성수혈부작용을진단할수있었고, 환자는해당항원음성적혈구제제를수혈하고특이적인합병증없이퇴원하였다. References 1. Olivès B, Mattei MG, Huet M, Neau P, Martial S, Cartron JP, et al. Kidd blood group and urea transport function of human erythrocytes are carried by the same protein. J Biol Chem 1995;270: Allen FM, Diamond LK, Niedziela B. A new blood-group antigen. Nature 1951;167: Plaut G, Ikin EW, Mourant AE, Sanger R, Race RR. A new blood-group antibody, anti Jkb. Nature 1953;171: Velasco Rodríguez D, Pérez-Segura G, Jiménez-Ubieto A, Rodríguez MA, Montejano L. Hemolytic disease of the newborn due to anti-jkb: case report and review of the literature. Indian J Hematol Blood Transfus 2014;30: Choi SY, Kim SI, Cho HI. Study on gene frequencies of blood groups in Koreans. Korean J Hematol 1984;19: Park DK, Kim YM, Bae CW, Choi YM, Lee WI. A case of hemolytic disease in a newborn due to anti-jkb. J Korean Pediatr Soc 2003;46: Shin JW. Unexpected red cell antibody detection by conditional combination of LISS/Coombs and NaCl/Enzyme gel tests at a tertiary care hospital in Korea: a 5-year study. Blood Res 2013;48: Choi MS, Cho YG, Lee J, Kim DS, Lee HS, Choi SI. Two cases of anti-jka whose reactivity was disappeared after enzyme treatment. Korean J Blood Transfus 2012;23: Shin JW, Chai GR, Bang HI, Park RJ, Choi TY. A case of anti-jka whose reactivity was abolished in an enzyme gel test. Korea J Blood Transfus 2009;20: Fudenberg H, Allen FH Jr. Transfusion reactions in the absence of demonstrable incompatibility. N Engl J Med 1957;256: Park TS, Kim HH, Son HC, Kim BC. Hemolytic transfusion reaction with acute renal failure due to anti-jkb : a case study. Korea J Blood Transfus 2002;13: Kim KS, Han JH, Chu WS, Kim HO. A case of intravascular hemolytic transfusion reaction due to anti-jkb. Korea J Blood Transfus 2001;12: Lown JA, Holland PA, Barr AL. Inhibition of serological reactions with enzyme-treated red cells by complement binding alloantibodies. Vox Sang 1984;46: Lown JA, Holland PA, Barr AL. A serum factor which inhibits serologic reactions with enzyme-treated red blood cells. Transfusion 1982;22: Lee DD, Oh SH, Shin HJ, Chung JS, Cho GJ, Park TS, et al. Diagnostic usefulness of antibody identification test including enzyme method. Korean J Hematol 2006;41:

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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,