혈액형은 잘 알려진 ABO system (A, B, O, AB) 뿐만 아니라 수백가지의 종류가 알려져 있다. 혈액형이란 단순히 말해서, RBC 표면에 발현되어있는 항원에 의해서 결정되기 때문이다.
오늘은 임상 현장에서 비교적 흔히 관찰할 수 있는 Lewis 혈액형에 대하여 알아보겠다.
Lewis 혈액형과 비예기 항체
수혈을 위해 시행하는 antibody screening test에서 양성이 나오게되면, antibody identification test를 추가적으로 시행하여 어떠한 종류의 비예기항체가 있는지 동정하게 된다. 이러한 비예기항체가 있는 경우에는 이 항원과 결합하는 항원을 가진 혈액을 수혈할 경우 용혈반응을 일으킬 수 있기 때문에, 혈액은행에서 혈액을 출고하기 전에 이 결과를 확인하고 해당 항원이 없는 혈액을 출고하여 수혈 사고를 예방하게 된다.
Lewis 혈액형에서, Anti-Lea, anti-Leb는 혈액은행 검사실에서 꽤나 자주 동정되는 비예기항체이다. 즉, 이 항체는 Lea항원과 Leb항원에 대한 항체이다. 다른 혈액형과 다르게 Lewis antigen은 적혈구로부터 생성되지 않고 plasma에 존재하는 glycolipid로부터 적혈구막에 흡착되는 형태로 항원성을 나타나게 된다. 특히 GI tract는 plasma Lewis glycolipid의 주된 공급원이다.
Lewis antigen의 합성
위와 같은 다소 복잡해보이는 과정에 의해 Lewis antigen이 합성된다. Lewis antigen의 합성은 ABO항원을 나타내는 ABH와의 공통적인 전구체인 type 1 precursor에서 시작된다. 이것은 galactose와 N-acetylglucose가 베타1->3 결합으로 연결되어 있는 구조이다.
여기에 FUT2와 FUT3에 의해 인코딩된 fucosyltransferase의 작용에 의해 Lewis antigen type이 결정된다. type 1 precursor에 FUT3가 작용하면 Lewis a (Lea) 항원이 생성되고, type 1 Precursor가 일단 FUT2의 작용에 의해 type 1 H antigen이 된 다음, 여기에 FUT3가 작용하면 Lewis b (Leb)항원이 생성된다.
따라서 FUT2, FUT3의 존재 유무에 따라 Lewis antigen의 표현형이 결정되는데, Le(a+b-), Le(a-b+), Le(a-b-), Le(a+b+)가 된다. 이중 Le(a+b+) 표현형은 드물지만 영아들에서 일시적으로 관찰될 수 있으며, 아시아계에서 많이 나타난다.
Lewis 항원에 대한 비예기 항체
이들 Lewis antigen에 대한 antibody는 IgM isotype이며 자연적으로 생기게 된다. IgM type 항체여서 placenta를 통과할 수 없기 때문에 HDFN(Hemolytic disease of the fetus and newborn)의 원인이 아니다. Lewis 항원은 신생아 적혈구에서 제대로 발현되지 않으며 많은 신생아는 Le(a-b-)로 타이핑 된다.
임신기간동안에는 circulating plasma volume의 증가, lipoprotein이 증가하므로 일시적으로 Le(a-b-)로 타이핑될 수 있으며, 이때 Lewis antibody가 존재할 수 있다.
Lewis antibody는 대체적으로는 임상적으로 크게 유의미하지는 않다. 대부분의 환자에서 Lewis 항원이 음성인 적혈구를 수혈할 필요가 없으며, ABO항원과 달리 Lewis 항원은 외인성 당지질 항원이므로 수혈 후 수일 이내에 수혈된 적혈구에서 쉽게 용출되고 탈락된다.
그렇지만, 예외적으로 Lewis antibody에 의한 용혈 수혈반응이 보고되기도 한다. 국내에서도 anti-Lea에 의한 용혈성 수혈반응이 보고된 적이 있다(Korean J Hematol 1989;24:27-33). 따라서, Lea 항원과 Leb 항원에 대한 비예기 항체가 검사에서 발견된다면, 가급적 Lea 항원과 Leb항원이 없는 혈액을 출고하는 수혈 전략이 적절할 것으로 생각된다.
잘 봤습니다 다양한 혈액형이 있군요
ABO혈액형말고도 수많은 혈액형이 있답니다.
нужна медицинская справка
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