Knull 표현형에서 항 Ku 항체로 인한 용혈수혈부작용 1예: 국내 첫 보고 = A Hemolytic Transfusion Reaction due to Anti-Ku Antibody in a Patient with Knull Phenotype: The First Case in Korea

다국어 초록 (Multilingual Abstract)

Knull phenotype completely lacks all Kell system antigens. Anti-Ku antibody is seen in immunized persons with Knull phenotype by transfusion or pregnancy. It can cause a fatal hemolytic transfusion reaction. A 66-yr-old male patient with liver cirrhosis visited emergency center due to acute
bleeding. The patient was at hypovolemic shock status: his blood pressure was 80/50 mmHg, pulse rate was 110/min and hemoglobin level was 4.4 g/dL. Because of the presence of antibody against high incidence antigen, we could not find any compatible blood for the patient. Nevertheless, 4 units
of packed RBCs had to be transfused. Moderate hemolytic transfusion reaction was developed after transfusion. At endoscopic examination, blood was spurting from gastric cardiac varix. Endoscopic histoacryl injection was tried, and bleeding was successfully controlled. After bleeding stopped, he
was managed for anemia using steroid and other medical therapy instead of transfusion. His hemoglobin level was improved to 7.7 g/dL at the time of discharge. Later he has been proved to have a Knull phenotype, which is very rare, and anti-Ku antibody. This report is the first case of anti-Ku in a
Knull phenotype person in Korea, who experienced a moderate hemolytic transfusion reaction.

국문 초록 (Abstract)

Knull 표현형에서는 Kell 혈액형군의 모든 항원이 결핍되어
있다. 항 Ku 항체는 Knull 표현형인 사람에서 수혈이나 임신 등
에 의하여 감작되어 생성되며, 치명적인 용혈수혈부작용을 일으
킬 수 있다. 간경변의 과거력이 있는 66세 남자 환자가 급성출혈
로 응급실로 내원하였다. 환자는 혈액량 감소성 쇼크 상태로 혈
압 80/50 mmHg, 맥박 110/분, 혈색소수치 4.4 g/dL였다. 고
빈도 항원에 대한 항체 때문에 환자에게 적합한 혈액을 찾을 수
없었으나, 당시 급박한 상황으로 4단위의 농축적혈구를 수혈
받은 후에 중등도의 용혈수혈부작용이 나타났다. 내시경검사상
위분문부의 정맥류 출혈이 있었으며, histoacryl 주입을 통한
내시경적 지혈술을 받은 후 성공적으로 지혈되었다. 출혈이 멈
춘 후에는 빈혈치료를 위하여 수혈대신 스테로이드와 기타 약
물치료를 실시하여, 퇴원 시에는 혈색소수치가 7.7 g/dL까지
상승하였다. 퇴원 후에 국내에서는 아직 보고가 없는 매우 드문
Knull 표현형 환자의 항 Ku 항체로 동정되었으며, 이로 인하여
중등도의 용혈수혈부작용을 경험하였기에 보고하는 바이다.

참고문헌 (Reference)

1 윤석호, "저결합성 항-Yka 항체가 검출된 1예-국내 첫 보고" 19 : 57-62, 2008

2 한규섭, "수혈의학. 제3판" 고려의학 226-227, 2006

3 Marconi M, "regional rare blood donor registry and bank of frozen blood units in ITALY" 76 : 25-, 2008

4 Manoura A, "Use of recombinant erythropoietin for the management of severe hemolytic disease of the newborn of a K0 phenotype mother" 24 : 69-73, 2007

5 Lee S, "The Kell blood group system: Kell and XK membrane proteins" 37 : 113-121, 2000

6 Redman CM, "The Kell blood group system and the McLeod Phenotype" 30 : 209-218, 1993

7 Brecher ME, "Technical manual. 15th ed" American Association of Blood Banks 340-343, 2005

8 Yu LC, "Molecular basis of the Kell-null phenotype: a mutation at the splice site of human KEL gene abolishes the expression of Kell blood group antigens" 276 : 10247-10252, 2001

9 Beadling WV, "Immunohematology, in Clinical diagnosis and management by laboratory methods. 21st ed" WB Saunders 633-636, 2006

10 Rolih SD, "High-titer, low-avidity (HTLA) antibodies and antigens: a review" 3 : 128-139, 1989

11 Lin M, "Fatal hemolytic transfusion reaction due to anti-Ku in a Knull patient" 19 : 19-21, 2003

12 정희정, "Anti-Jra 항체 양성 환자에게 Jra 항원 양성 적혈구 수혈 경험" 18 : 111-115, 2007