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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2021.1
- 수록면
- 183 - 190 (8page)
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초록· 키워드
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T세포큰과립림프구백혈병(T-cell large granular lymphocytic leukemia, T-LGLL)은 드물게 이식후림프증식질환(post-transplant lymphoproliferative disorder, PTLD)의 형태로 발병한다. 본 연구에서는 T-LGLL PTLD 58예(본 논문 2예와 문헌증례 56예)의 임상병리학적 소견을 기술하고, 고형장기이식(solid organ transplantation, SOT)과 동종조혈모세포이식(allogeneic hematopoietic stem cell transplantation, alloHSCT) 환자군에서의 특성을 비교하였다. 첫째 증례는 39세 남자로 간이식 후 81.7개월 경과 시점에 T-LGLL로 진단되었다. 진단 당시 거대세포바이러스(cytomegalovirus, CMV) 대장염 소견을 동반하였다. 일차면역결핍증을 기저질환으로 가지고 있었으며,?IKZF1?생식세포 변이 양성이었다. 둘째 증례는 51세 남자로 ALL로 alloHSCT 시행 후 1.8개월에 T-LGLL로 진단되었다. CMV DNA 양성이었고, 질환은 공여자 기원이었다. 두 증례 모두 마지막 추적일까지 생존해 있었으나 림프구증가증은 지속되고 있었다. 전체 환자군 분석에서 이식 후 T-LGLL 발병까지의 소요기간의 중앙값은 43.2개월이었고, 큰과립림프구 수의 중앙값은 2.5×109/L이었다. 절반에 가까운 환자(46.0%)에서 CMV 양성 소견이 확인되었다. 임상경과가 기술된 환자들 중 대부분(80.0%)은 양호한 결과를 보였다. SOT에 비해 alloHSCT 환자군에서 T-LGLL까지의 소요기간은 유의하게 짧았고(P<0.001), CMV 양성률은 높은 경향을 보였다(P=0.067), 자가면역질환과의 연관성은 11예의 환자(SOT 환자 9예, alloHSCT 환자 2예)에서 기술되어 있었다. 본 증례분석 연구는 이식 초기 CMV 재활성화가 T-LGLL의 타당한 유발원인임을 시사해 주고, 특히, 자가면역질환의 특성을 보이는 SOT 환자에서는 T-LGLL의 발병 가능성을 고려해야 함을 보여준다.
목차
등록된 정보가 없습니다.
참고문헌 (20)
참고문헌 신청
Lamy T / 2017 / LGL leukemia : from pathogenesis to treatment / Blood 129:1082~1094
Herreman A / 2013 / Clinicopathological characteristics of posttransplant lymphoproliferative disorders of T-cell origin : single-center series of nine cases and meta-analysis of 147 reported cases / Leuk Lymphoma 54:2190~2199
Walter JE / 2019 / Autoimmunity as a continuum in primary immunodeficiency / Curr Opin Pediatr 31:851~862
Hoshino A / 2017 / Abnormal hematopoiesis and autoimmunity in human subjects with germline IKZF1 mutations / J Allergy Clin Immunol 140:223~231
Awada H / 2020 / Large granular lymphocytic leukaemia after solid organ and haematopoietic stem cell transplantation / Br J Haematol 189:318~322
Herreman A / 2013 / Clinicopathological characteristics of posttransplant lymphoproliferative disorders of T-cell origin : single-center series of nine cases and meta-analysis of 147 reported cases / Leuk Lymphoma 54:2190~2199
Walter JE / 2019 / Autoimmunity as a continuum in primary immunodeficiency / Curr Opin Pediatr 31:851~862
Hoshino A / 2017 / Abnormal hematopoiesis and autoimmunity in human subjects with germline IKZF1 mutations / J Allergy Clin Immunol 140:223~231
Awada H / 2020 / Large granular lymphocytic leukaemia after solid organ and haematopoietic stem cell transplantation / Br J Haematol 189:318~322
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