간이식 후 발생한 림프구 증식성 질환의 임상적 양상 = Clinical Manifestations of Posttransplantation Lymphoproliferative Disorder (PTLD) after Liver Transplantation

다국어 초록 (Multilingual Abstract)

Background : Post-transplantation lymphoproliferative disorder (PTLD) after liver transplantation is a rare but potentially fatal disease. Clinical manifestations and prevalence of PTLD after liver transplantation in Korea have not been investigated thoroughly.
Materials and Methods : A retrospective chart review was done for 284 liver transplant recipients at Samsung Medical Center, Seoul, Korea during the period from 1996 to 2003.
Results : The incidence of PTLD after liver transplantation was 3.9% (11/284). PTLDs were more prevalent in children (9/55, 16.4%) than in adults (2/237, 0.9%; P<0.01). Among the PTLD patients, four cases were male (36.3%) and seven were female (63.7%). Median time from the transplantation to PTLD diagnosis was 9 months. The type of PTLD was as follows : early lesion (6 cases, 54.5%), polymorphic PTLD (3 cases, 27.3%), and B cell lymphoma (2 cases, 18.2%). PTLDs were more prevalent in the patients with cyclosporine use (OR 13.28, 95% CI : 1.29-136.31, P=0.03), acute rejection (OR 5.63, 95% CI : 1.03-30.62, P=0.04), and negative serology for EBV VCA IgG (OR 19.15, 95% CI : 1.99-183.98, P=0.01) by multivariate logistic regression. Three patients (27.3%) died of B cell lymphoma (2 cases) and polymorphic PTLD (1 case). The remaining patients were improved with reduction of immunosuppression and treatment with acyclovir.
Conclusion : The incidence of PTLD was high in children. The risk factors of PTLD were negative serology for EBV VCA IgG, history of acute rejection, and cyclosporine use. Considering the poor prognosis of PTLD, effective strategies for prevention and early diagnosis for early treatment should be emphasized.

국문 초록 (Abstract)

목 적 : 이식 후 발생하는 림프구 증식질환 (Post- transplantation lymphoproliferative disorder, PTLD)은 환자에게 치명적일 수 있는 합병증이지만 국내에서 간이식 후 PTLD 보고례가 많지 않아 이에 대한 임상적인 양상을 분석하였다.재료 및 방법 : 삼성서울병원 이식외과에서 1996년부터 2003년까지 간이식을 시행받은 총 284명의 환자들을 대상으로 후향적 연구를 진행하였다.결 과 : 총 284명의 간이식례 중 PTLD가 발생한 경우는 11례로 3.9%의 발생률을 보였다. 성인의 발생률은 0.9%, 소아의 발생률은 16.4%로 소아에서 발생률이 높았다. 남자가 4명, 여자가 7명이었고 이식 후 PTLD 발생까지의 중앙값은 9개월이었다. PTLD의 유형으로는 초기 병변이 6례, polymorphic PTLD가 3례, B cell lymphoma가 2례였으며 B cell lymphoma 환자 2명과 polymorphic PTLD 환자 1명은 사망하였다. PTLD의 발생과 관련된 요인으로는 cyclosporine 사용(OR 13.28, 95% CI : 1.29- 136.31, P=0.03), 급성 거부반응(OR 5.63, 95% CI : 1.03- 30.62, P=0.04), EBV-VCA IgG 음성인 환자(OR 19.15, 95% CI : 1.99-183.98, P=0.01)에서 발생빈도가 높았다.

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