ABO 양방향 부적합 신장이식 후 발생한 일과성림프구증후군 1예 보고
A Case Report of Passenger Lymphocyte Syndrome Following Bidirectional ABO-mismatched Kidney Transplantation
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일과성림프구증후군(passenger lymphocyte syndrome)은 이식 편대숙주병의 아형으로, 공여자에서 유래한 B 림프구가 수혜자의 적혈구에 대한 항체를 생성하여 용혈성 빈혈을 일으키는 질환이다. 일과성림프구증후군의 진단은 직접항글로불린(direct antiglob ulin test) 검사 양성 소견을 비롯하여 용혈성 빈혈을 시사하는 소견과, 원인이 되는 공여자 유래 항체의 동정을 포함한다. 치료는 보 존적 치료를 주로 하며, 공여자 혈액형의 적혈구 수혈과 면역억제제 투여, 그리고 필요시 혈장교환술을 시도해 볼 수 있다. 만성 신부전 상태로 고혈압, 관상동맥질환의 과거력을 가진 64세 남성이 ABO 양방향 불일치 신장이식을 받았다. 수혜자의 혈액형은 A+형이었으며, 공여자의 혈액형은 B+형이었다. 이식 후, 환자의 혈색소 수치가 감소하였으며, A형 적혈구 수혈에도 불구하고 혈색소 수치는 일시적으로 증가하였다가 다시 감소하였다. 수술 후 25일째, 혈색소 수치는 6.3 g/dL까지 감소하였다. 젖산탈수소효소 수치의 증가와 직접항글로불린검사 결과 양성, 항체용출검사상 anti-A가 검출되어 일과성림프구증후군이 원인으로 추정되었다. 이후 적혈구 수혈을 A형에서 O형으로 전환하였으며, 혈장교환술을 시행하였고 혈색소 수치는 다시 회복하여 수술 후 35일째 특이 합병증 없이 퇴원하였다. 본 증례는 ABO 양방향 불일치 장기이식에서의 일과성림프구증후군 증례로서 한국에서 첫 보고이다
Abstract
Passenger lymphocyte syndrome (PLS) is a subtype of graft-versus-host disease caused by the production of antibodies by transferred donor B lymphocytes against red blood cells (RBCs) of the recipient. A 64-year-old male diagnosed with stage five chronic kidney disease underwent bidirectional ABO-mismatched kidney transplantation. The blood groups of the recipient and the donor were A+ and B+, respectively. After transplantation, hemoglobin (Hb) levels were eventually reduced to 6.3 g/dL on postoperative day (POD) 25. Elevated lactate dehydrogenase levels and positive direct antiglobulin test results indicated immune-mediated hemolysis as a possible cause of anemia. After switching RBC transfusion from type-A to type-O and conducting plasma exchange four times, on POD 35, Hb levels of the patient recovered up to 9.1 g/dL, and the patient was discharged from the hospital without complications. To the best of our knowledge, PLS in a bidirectional ABO-mismatched solid organ transplantation has been reported once in India, and this is the first case to be reported in Korea.
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