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소아 간이식에서 Posttansplant Lymphoproliferative Disorder (PTLD): 삼성서울병원의 경험
최연호,이석구,서정민,조재원,김성주,이광웅,박제훈,고영혜,권기영,Choe, Yon-Ho,Lee, Suk-Koo,Seo, Jeong-Meen,Joh, Jae-Won,Kim, Sung-Joo,Lee, Kwang-Woong,Park, Je-Hoon,Ko, Young-Hye,Kwon, Ki-Young 대한소아소화기영양학회 2003 Pediatric gastroenterology, hepatology & nutrition Vol.6 No.1
목 적: EBV의 초감염과 면역억제의 강도는 소아 간이식에서 PTLD 발생의 주요 위험인자로 알려져 있다. 삼성서울병원에서 6년간 경험한 PTLD 5례의 임상양상을 분석해보고자 한다. 대 상: 1996년부터 2002년 6월까지 41례의 소아간이식 환아를 대상으로 하였는데 이중 7명이 사망하였고, PTLD로 사망한 1명을 포함한 35명을 대상으로 분석하였다. 방 법: 공여자의 EBV 항체가 양성이고 수혜자가 음성일 경우 고위험군으로, 그 외의 경우를 저위험군으로 정의하였다. 위험군 분류, 면역억제제 종류, 간이식이 행하여진 나이, 수술 후 PTLD 진단까지의 기간, 수술 후 EBV 항체의 양전 시기, 거부반응에 대한 치료 여부, PTLD의 증상들인 발열, 설사, 빈혈, 장출혈 식욕부진 저알부민혈증 등의 발현 여부를 후향적으로 조사하였다. 결 과: 1) PTLD는 전체 소아 간이식 환아 41명 중 5명에서 발생하였다(12.2%). 고위험군은 16명으로 이 중 5명(31.3%)에서 PTLD가 발생하였고 저위험군 19명중에서는 한 명도 발생하지 않았다. 2) 사망례를 제외한 4명은 tacrolimus로 면역억제 치료를 받았으며 수술 당시 나이는 평균 10.8개월이었고 수술 후 PTLD 진단까지 평균 9.8개월이 걸렸 다. EBV 양전 시기는 수술 후 평균 6개월이었다. 3) 사망례를 포함한 5명 중 3명에서 PTLD 진단 이전에 간이식 거부반응이 있었고 스테로이드 pulse 치료 등 면역억제를 증가시키는 치료를 행하였었다. 4) 한 명에서 쉰 목소리 증상이 발견되어 조직검사 상 후두 PTLD와 장 PTLD로 진단하였고 나머지 네 명은 모두 혈변이 있었으며 대장 조직검사 상 장 PTLD로 진단하였다. 5) 빈혈과 저알부민 혈증은 모든 환아에서 나타나고 있었고 발열, 설사, 장출혈이 5명 중 4명에서 보였으며 식욕부진은 5명 중 3명에서 나타났다. 결 론: 10%가 넘는 발병률을 보이는 PTLD는 소아 간이식 후 매우 중요한 합병증으로서 특히 EBV의 고위험군에서 31%에 이르는 발병률을 가지고 있는 바 이들에 대하여 EBV 상태와 면역억제 유지에 대한 철저한 대비가 있어야 할 것으로 생각된다. 특히 빈혈, 저알부민혈증, 발열, 설사, 장출혈 등이 동반할 때에는 PTLD를 의심해 볼 수 있다. PTLD의 공통적인 발병양상으로는 첫째, 공여자가 EBV 양성이고 수혜자가 음성이면서, 둘째, EBV가 수술 후 6개월 경 양전되며, 셋째, 1세 전후에 수술을 시행하고 넷째, 간이식 거부반응을 겪으면서 면역억제의 강도가 높아졌을 경우 등이다. Purpose: In a retrospective study for the pediatric patients who underwent liver transplantation in the past 6 years at Samsung Medical Center, the clinical features of 5 patients with posttransplant lymphoproliferative disorder (PTLD) were analyzed. Methods: Between June 1996 and June 2002, 41 pediatric patients underwent liver transplantation. Seven of them died in the postoperative period. Thirty-five including one patient who died of PTLD were finally reviewed. Patients were divided into two groups: high risk group, EBV naive recipients of EBV-positive grafts; low risk group, the patients other than those in high risk group. The authors reviewed age at operation, immunosuppressive agent, postoperative duration until diagnosis, postoperative duration until EBV seroconversion, presence of treatment against rejection, and presenting symptoms of PTLD. Results: Five of 41 patients (12.2%) developed PTLD. All of them belonged to high risk group, and the incidence of PTLD in high risk group was 31.3% (5/16). The mean age at operation was 10.8 months old and the mean duration between operation and diagnosis for PTLD was 9.8 months. Primary EBV infection developed after a median of 6 months after transplantation. One patient was diagnosed as laryngeal and gastrointestinal PTLD and the other four, gastrointestinal PTLD. The following symptoms and signs were seen in the patients: anemia (100%), hypoalbuminemia (100%), fever (80%), diarrhea (80%), gastrointestinal bleeding (80%), and anorexia (60%). Conclusion: PTLD is one of the major complications after pediatric liver transplantation, especially in the group of high-risk recipients. Anemia, hypoalbuminemia, fever, diarrhea and gastrointestinal bleeding were features that are characteristic of PTLD. The common features of PTLD development were: (i) EBV-positive donors placed into EBV naive recipients, (ii) primary EBV infection about 6 months after transplantation, (iii) young age, about 1 year old at operation, and (iv) the requirement for intensive posttransplant immunosuppression.
최명섭 ( Myung Sub Choi ),이현섭 ( Hyunsup Lee ),권혁철 ( Hyuk Chul Kwon ),배문환 ( Moon Hwan Bae ),고영혜 ( Young Hye Ko ),김희진 ( Hee Jin Kim ),이범세 ( Beom Se Lee ),구본경 ( Bon Kyung Koo ) 대한임상검사과학회 2015 대한임상검사과학회지(KJCLS) Vol.47 No.4
A bone marrow biopsy that has undergone decalcification with 10% nitric acid could not be used for various pathological tests and had extremely limited reproducibility. The fixing solution of each experimental group was differentiated in usage, one solution including acid and the other not. The use of the decalcification solution was separated into either acidic or alkaline (EDTA), and further experiments were conducted with differing time phases. When using a fixing solution and decalcification solution which included acid, the specimens were faulty to the extent that all pathological tests were impossible. However specimens that were processed with an EDTA type decalcification solution did not display a non-specific reaction in EBV ISH and were even able to produce results that were at a level suited to various studies or a pathological diagnosis in the FISH, DNA, RNA tests. By improving the fixing and decalcification of bone marrow biopsy, the study was able to make possible ISH, FISH, DNA tests as well as RNA study, and secured the sensitivity, specificity, and reproducibility of various test methods. The stabilization of various test methods that use bone marrow biopsy contributes to the diagnosis, prognosis, prediction, treatment of the patient and provide guidelines for decision-making.
Pembrolizumab 투여로 Pseudoprogression 이후 완전 관해에 도달한 호지킨 림프종 1예
홍주현 ( Joohyun Hong ),배주환 ( Joohwan Bae ),이상글 ( Sang Geul Lee ),임민기 ( Mingi Lim ),고영혜 ( Young Hye Ko ),김석진 ( Seok Jin Kim ),김원석 ( Won Seog Kim ) 대한내과학회 2017 대한내과학회지 Vol.92 No.4
Classical Hodgkin lymphoma (cHL) is a highly curable disease, but the prognosis for relapsed/refractory cHL is grave. Pembrolizumab has recently shown impressive effects in patients with relapsed/refractory cHL in a phase Ib study (KEYNOTE-013). This report presents a case of a 17-year-old male with refractory cHL who received multiple chemotherapy regi-mens and radiotherapies, including brentuximab vedotin. Following both the second and fourth cycles of intravenous pem-brolizumab 100 mg (2 mg/kg), positron emission tomography/computed tomography (PET/CT) scan showed progression. However, because performance status and fever improved, treatment was continued, and complete remission was confirmed by PET/CT after eight cycles of pembrolizumab. This case suggests that clinicians need to be aware of the potential for pseudoprog-ression in patients treated with pembrolizumab. (Korean J Med 2017;92:415-418)
증례 : 혈액종양 ; 임신과 동반된 원발성 종격동 거대 B세포 림프종에서 Rituximab 치료
김은경 ( Eun Kyoung Kim ),김석진 ( Seok Jin Kim ),박성범 ( Sung Bum Park ),조진현 ( Jin Hyun Cho ),고영혜 ( Young Hye Ko ),김원석 ( Won Seog Kim ) 대한내과학회 2011 대한내과학회지 Vol.80 No.2S
저자들은 임신 중기에 진단된 PMLBCL 환자를 성공적으로 치료하였으며, 중기 및 후기 임신과 관련한 R-CHOP 복합 항암화학요법의 효과와 안전성을 입증하였다. 본 증례는 우리나라에서 임신 여성에게 시행한 R-CHOP 요법의 사용에 대한 첫 보고임에 의의를 두는 바이다. A 33-year-old woman developed primary mediastinal large B-cell lymphoma (PMLBCL) during the second trimester of pregnancy. She was immediately treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP). After the third cycle of R-CHOP, she delivered a healthy baby by Caesarian section. She was in complete response after she finished all courses of treatment, and the rituximab-containing chemotherapy during pregnancy induced no adverse effect in the fetus. Thus, R-CHOP appears to be an effective, safe treatment option for PMLBCL encountered during the second and third trimesters of pregnancy. To our knowledge, this is the first report of the use of rituximab plus CHOP in a pregnant woman in Korea. (Korean J Med 2011;80:S273-S277)