증례 : 혈액종양 ; 동종 조혈모세포이식 후 다발성 근염으로 발현된 만성 이식편대숙주반응

이혜원 ( Hye Won Lee ),최희경 ( Hee Kyung Choi ),김수정 ( Soo Jeong Kim ),황도유 ( Doh Yu Hwang ),최준용 ( Jun Yong Choi ),정준원 ( June Won Cheong ),민유홍 ( Yoo Hong Min ) 대한내과학회 2009 대한내과학회지 Vol.76 No.1

동종 조혈모세포이식 후의 합병증으로 드물게 다발성근염으로 만성 이식편대숙주반응이 생길 수 있으며, 경우에 따라 치명적일 수 있으므로 조기 진단이 중요하겠다. 그러나, 임상 양상이 비특이적일 경우 진단이 쉽지 않으며 이식 후 수년 뒤에도 유일한 이식편대숙주반응으로 발현될 수 있어 동종 조혈모세포이식을 받았던 환자를 진료할 때에는 이에 대한 주의가 필요하겠다. Chronic graft-versus-host disease (cGVHD) remains one of the major complications of allogeneic hematopoietic stem cell transplantation. Although cGVHD has various manifestations in almost all organs, cases of cGVHD involving skeletal muscle are rare. We experienced a 26-year-old man with polymyositis with no other concurrent cGVHD after HLA-matched myeloablative transplantation for acute myelogenous leukemia. He had a history of acute and chronic GVHD. The patient complained of fever and myalgia 3 years after transplantation. The serum creatine kinase (CK, 2,223 IU/L) and aldolase (87.6 sigmaU/mL) were elevated. The muscle biopsy and electromyographic findings were consistent with myositis with necrosis. His condition improved dramatically with immunosuppressive therapy. Although muscle involvement, alone, in cGVHD is very rare, early diagnosis and proper treatment are still important. (Korean J Med 76:110-113, 2009)

만성골수성백혈병 한국 치료 가이드라인-대한혈액학회

손상균 ( Sang Kyun Sohn ),정철원 ( Chul Won Jung ),조덕연 ( Deog Yeon Jo ),정준원 ( June Won Cheong ),대한혈액학회만성골수성백혈병연구회 ( Chronic Myelogenous Leukemia Working Party The Korean Societ ),김대영 ( Dae Young Kim ),이정옥 ( 대한내과학회 2015 대한내과학회지 Vol.88 No.4

Background/Aims: The first edition of the Korean treatment guidelines for chronic myelogenous leukemia (CML) was published in 2006. We intend to update those guidelines to include the use of next-generation tyrosine kinase inhibitors (TKIs). Methods: New guidelines were developed in 2012 based on the results of a survey and a consensus meeting of various Korean experts, the reports of recent clinical studies, and updated guidelines from external study groups. Results: An assessment of risk factors is strongly recommended before treating newly diagnosed chronic phase CML. Imatinib, dasatinib, and nilotinib are reimbursable in Korea as first-line treatments, and the patient`s age, comorbidities, and possible adverse events should be considered in the choice of treatment. Molecular studies are recommended for assessing treatment efficacy instead of invasive cytogenetic response evaluations, and an early response is believed to correlate with a good prognosis. Second-line TKIs can be considered for patients who fail or are intolerant of first-line therapy, pending analysis of ABL tyrosine kinase mutation status. For treating advanced stages, a combination of TKIs with cytotoxic agents and hematopoietic cell transplantation is recommended. The adverse effects of TKI therapy can be managed via dose reduction and supportive care, or switching to an alternate TKI. Conclusions: The use of TKIs has improved the outcome of CML treatment. Treatment-free remission after discontinuing TKIs might be possible in select patients who achieve sufficient response, indicating that curative treatment for CML can be expected in the future.

의과대학 수업수준에서의 준거설정 사례 연구

이영주 ( Lee Yeongju ),박장희 ( Park Janghee ),정준원 ( Cheong June-won ),김수정 ( Kim Soo Jeong ),김윤덕 ( Kim Yundeok ) 인하대학교 교육연구소 2017 교육문화연구 Vol.23 No.2

역량중심교육은 학생교육의 질적 강화 및 구체적인 교육효과를 요구하고 있다. 이를 위해서는 적절한 성취수준 설정 및 학생의 도달 여부를 확인하는 준거참조평가(절대평가)가 보다 적절할 것이다. 준거참조평가를 위해서는 준거설정방법을 통해 각 성취수준을 구분하는 분할점수를 산출해야 한다. 기존 준거설정방법들은 대부분 고부담시험에 적용되었으나, 대학의 수업수준에서 이뤄진 연구는 거의 없었다. 본 연구는 준거참조평가를 시행하는 의과대학의 실제 수업에서 활용하는 준거설정방법을 소개하였다. 이를 위해 준거설정방법 중 Modified Angoff 방법과 Modified Ebel 방법을 이용하여 성취수준별 분할점수를 산출했다. 산출결과 Modified Ebel 방법이 Modified Angoff 방법보다 엄격하게 평정되어 분할점수가 높았다. 또한 Modified Ebel 방법은 예상정답률과 실제정답률을 사용했을 때 분할점수에 차이가 있었으며, 실제정답률을 반영하는 것이 보다 현실적이었다. 본 연구는 고부담시험에서 주로 이뤄지는 준거설정을 실제 의과대학에서 사용하는 방법으로 의과대학 성적에 적용해 보았다는데 의의가 있다. 창의성 기반의 역량중심교육을 보다 활성화하려면, 경쟁에 관심을 두는 규준참조평가보다는 협력학습을 가능하게 하는 준거참 조평가가 더욱 강조될 것이고, 이때 다양한 준거설정방법이 수업수준에 적용될 것으로 예측된다. Competency-centered education and the Fourth Industrial Revolution require qualitative strengthening of student education and concrete educational effects. To do this, it is appropriate to establish appropriate levels of achievement and to assess the criterionreferenced evaluation (so-called absolute evaluation). For the criterion-referenced evaluation, the division score between each grade should be calculated through the standard setting method. The existing standard setting methods were carried out in high-stakes examinations, but there were few studies conducted at the class level of universities. This study compares the standard setting method of the medical school which performs the criterion referenced evaluation in the actual class level. Among the standard setting methods, the modified score was calculated using Modified Angoff method and Modified Ebel method. As a result, Modified Ebel method was more strict than Modified Angoff method, and the Modified Ebel method had a higher cutoff score than the Modified Angoff method. It was more realistic to reflect actual difficulty. In this study, it is meaningful to apply the standard setting method, which is mainly used in the high stakes test, to the results of the medical college as a method used in actual medical college. In the future, competency-based evaluation will be more emphasized in order to activate competence-based education and creativity through cooperative learning rather than competition.

임신 중 동반된 급성 림프구성 백혈병

최현정 ( Hyun Jung Choi ),박용원 ( Yong Won Park ),정준원 ( June Won Cheong ),민유홍 ( You Hong Min ),권자영 ( Ja Yong Kwon ),권한성 ( Han Sung Kwon ),김영한 ( Young Han Kim ),김성훈 ( Sung Hoon Kim ) 대한산부인과학회 2005 Obstetrics & Gynecology Science Vol.48 No.2

The incidence of acute leukemia in pregnancy is estimated to be about 1 per 75000 pregnancies, and the incidence of lymphocytic leukemia is known to be lower than myelocytic leukemia. Pregnancy dose not affect the course of acute leukemia, but thrombocyto

급성림프구성백혈병 환자에서 막성 콩팥병증으로 나타난 만성 이식편대 숙주병 1예

정지예 ( Ji Ye Jung ),윤설희 ( Sul Hee Yoon ),황도유 ( Doh Yu Hwang ),정준원 ( June Won Cheong ),김진석 ( Jin Seok Kim ),정현주 ( Hyun Joo Jung ),민유홍 ( Yoo Hong Min ) 대한내과학회 2008 대한내과학회지 Vol.75 No.1S

증례 : 혈액종양 ; 이식초기의 급속한 림프구증식을 동반한 치명적 PTLD 증례

이정연 ( Jung Yeon Lee ),김규리 ( Gyuri Kim ),이은영 ( Eunyoung Lee ),박세희 ( Se Hee Park ),김진석 ( Jin Seok Kim ),정준원 ( June Won Cheong ),민유홍 ( Yoo Hong Min ) 대한내과학회 2015 대한내과학회지 Vol.89 No.2

Post-transplant lymphoproliferative disorder (PTLD) is a spectrum of clinically and morphologically heterogeneous lymphoid proliferations of various clonal compositions that are observed after hematopoietic stem cell transplantation (HSCT) or solid organ transplantation. The majority of PTLD cases are associated with Epstein-Barr virus (EBV) infection, while overt peripheral blood (PB) or bone marrow (BM) involvement in PTLD is uncommon in early lesions. We report a 38-year-old woman with EBV-related PTLD, with BM and PB involvement, who presented with peripheral lymphocytosis as an early lesion 1 month after haploidentical HSCT for Philadelphia chromosome-positive acute lymphoblastic leukemia was performed during first complete remission. Although PB or BM involvement of PTLD after HSCT is uncommon in early lesions, peripheral lymphocytosis can be an initial presenting manifestation of PTLD, as in this case. (Korean J Med 2015,89:243-248)

NK/T 세포 림프종 환자에서 시행된 비골수제거성 조혈모세포이식

정준원,김진석,장준호,서형찬,이승태,김현옥,양우익,민유홍,한지숙,고윤웅 대한조혈모세포이식학회 2001 대한조혈모세포이식학회지 Vol.6 No.2

저자 등은 맹장, 충수돌기 및 흉막을 침범한 고위험군 NK/T 세포 림프종 환자에서 비골수제거 조혈모세포이식을 시행한 후 이식연관 합병증 없이 무병생존하였기에 보고하는 바이다. NK/T cell lymphoma is a rare type of non-Hodgkin"s lymphomas. It has the histological features of angiocentric lymphoma characterized by prominent angio-invasion by tumor cells, and commonly presents in nasal and paranasal structures. Non-nasal form of NK/T cell lymphoma involves skin, gastrointestinal tract, or liver. With conventional chemotherapy and/or radiotherapy, NK/T cell lymphoma shows poor response rate and seldom results in long term survival. Especially, non-nasal form has more aggressive clinical manifestations and grave outcome than nasal form. We report a case of NK/T cell lymphoma presenting with small bowel perforation and malignant pleural effusion due to lymphoma infiltration, transplanted with allogeneic peripheral blood stem cells from HLA identical sibling donor after fludarabine-based nonmyeloablative chemotherapy. After transplantation, the patient achieved an enduring tri-lineage hematological complete response without any evidence of relapse for 10 months.

동종 조혈모세포이식 후 급성 이식편대 숙주반응 시 T 세포의 OX-40 표현

장준호,정준원,김진석,서형찬,이승태,민유홍,한지숙,고윤웅 대한조혈모세포이식학회 2000 대한조혈모세포이식학회지 Vol.5 No.2

배경:OX-40은 분자량이 50-kD인 당단백으로 최근 활성화되기 시작한 CD 4+ T 세포에 표현된다. 최근 쥐 모델에서 T 세포의 OX-40 표현과 급성 이식편대 숙주반응과의 높은 연관성이 보고되었으나, 아직까지 사람에 있어 급성 이식편대 숙주반응과 T세포의 OX-40 표현간에 의미 있는 연관성을 보고한 연구는 없는 실정이다. 이에 저자는 동종 조혈모세포이식 후 말초 혈액 내 T세포에서의 OX-40 표현을 측정하여 급성 이식편대 숙주반응과의 상관성 등 그 임상적 의의를 평가하고자 본 연구를 시행하였다. 방법:1998년 12월부터 1999년 9월까지 세브란스 병원에서 악성 혈액종양 및 재생불량성 빈혈로 동종 조혈모세포이식을 시행 받은 16예의 환자와 공여자를 대상으로 하였다. 환자들의 말초혈액은 동종 조혈모세포이식 2주전과 이식 후 10일, 17일, 24일째 채취하였다. 공여자의 말초혈액은 이식 2일 전 채취하였다. 세포는 단클론항체로 염색하여 유세포분석기로 분석하였다. OX-40 표현은 유세포분석기를 이용 정량화 하였으며 OX-40+ T 세포가 환자 혹은 공여자의 유래인지를 확인하기 위해 polymerase chain reaction (PCR)을 이용한 variable number of tandem repeats (VNTR) 검사를 시행하였다. 결과: 1) 대상 환자 16예의 평균 연령은 32세(17~50세)였다. 14예의 환자에서 혈연간 동종 조혈모세포이식이 시행되었고, 이 중 12예는 골수이식, 2예에서는 말초 조혈모세포이식을 시행하였다. 2예에서는 비혈연간 동종 조혈모세포이식을 시행하였다. 2) 급성 이식편대 숙주반응이 발생한 6예에서 CD 4+ T 세포의 OX-40 표현율은 평균 33.9±6.8% (22.1~53.2)로 급성 이식편대 숙주반응이 나타나지 않은 환자군에서의 28.2±7.1% (15.2~42.4)와 비교하여 통계학적으로 유의한 차이는 없었으며(p=0.18). 10일, 17일, 24일째에서도 통계학적으로 유의한 차이를 관찰할 수 없었다(p=0.12, p=0.14, p=0.22). 10예의 조혈모세포 공여자의 CD 4+ T세포의 OX-40 표현율은 평균 8.0 ± 4.6% (1.7~16.9%)였다. 3) CD 4+ T 세포의 OX-40 표현 유무 및 그 정도와 급성 이식편대 숙주반응의 중증도와 임상양상과는 유의한 상관관계를 관찰할 수 없었다. 4) 급성 이식편대 숙주반응의 치료에 따른 CD 4+ T 세포의 OX-40 표현 변화는 관찰되지 않았다(p=0.69). 5) 동종 조혈모세포이식 후 환자의 말초혈액에서 시행한 VNTR-PCR 결과 혼합 키메리즘을관찰할 수 있었다. 결론:동종 조혈모세포이식 후 CD 4+ T 세포에서의 OX-40의 표현은 정상인과 동종 조혈모세포이식 전에 측정한 OX-40 표현율과 비교하여 통계학적으로 유의한 상승을 관찰할 수 있었으나 급성 이식편대 숙주반응이 발생한 군에서의 표현율과 급성 이식편대 숙주반응이 나타나지 않았던 군 간에 통계학적으로 유의한 차이를 관찰할 수 없었고 급성 이식편대 숙주반응이 발생한 군에서 치료에 따른 CD 4+ T 세포에서의 OX-40 표현율의 통계학적 유의성을 관찰하지 못하여 동물실험에서와는 달리 급성 이식편대 숙주반응의 예측인자로써 유의한 연관성을 관찰할 수 없었고, 급성 이식편대 숙주반응의 치료를 판정하는 지표로써의 역할에서도 유의한 연관성을 관찰할 수 없었다. Background:Acute GRAFT-VERSUS-HOST disease (GVHD) is a major and often lethal consequence of allogeneic stem cell transplantation. The OX-40 molecule is a 50-kD glycoprotein that is expressed on recently activated CD4+ T cells. Although recently studies in rat model have suggested an close association between OX-40 expression on peripheral blood CD 4+ T cells and course of acute GVHD, there have been few studies in human yet. This study was performed to investigate whether the kinetics of repopulation of T cells in human recipients of HLA-matched allogeneic stem cell transplantation and their OX-40 expression would be predictive of acute GVHD and/or its response to immunosuppressive therapy. Methods:OX-40 expression on T cells was evaluated in 16 patients with hematologic malignancies or aplastic anemias, who received allogeneic stem cell transplantation between December 1998 and September 1999, at the Yonsei University College of Medicine, Severance Hospital. 10 donors were evaluated for control. Blood samples of the recipients were taken at day 14 before allogeneic stem cell transplantation and +10, +17, +24 after allogeneic stem cell transplantation. Blood samples of the donors were taken at day 2 before allogeneic stem cell transplantation. All staining was performed with monoclonal antibodies and OX-40, CD4, CD8 expression on lymphocytes was analyzed by flow cytometry. Mixed chimerism was determined by using the variable number of tandem repeats (VNTR) by polymerase chain reaction (PCR) in one patient. Results:OX-40 expression on CD4+ T lymphocytes of healthy donors and recipients before stem cell transplantation was with a mean percentage of 8.1±4.6%(1.7~16.9%), 15.5±7.8% (2.4~27.2%). OX-40 expression by CD8+ T lymphocytes of healthy donors and stem cell transplantation recipients was undetectable. The expression of OX-40 as a percentage of the CD4+ cells of recipients after stem cell transplantation was increased at day +10, day +17, day +24 compared with at day -14 (p=0.01, 0.02, 0.01, respectively). Six out of sixteen patients developed GVHD. OX-40 expression on CD4+ T lymphocytes of the patients with GVHD was 33.9±6.8%. But no difference in the kinetics of OX-40 expression by CD4+ T cells was observed between the patients that did or did not develop GVHD, nor did the clinical effect of any treatment given for GVHD correlate with alterations in OX-40 expression by CD4+ T cells. After stem cell transplantation, one patient showed mixed chimerism in peripheral blood cells by VNTR-PCR in one patient analyzed. Conclusion: Our observation do not support the view that OX-40 expression is predictive of GVHD or is a useful tool for monitoring response to GVHD. In addition, earlier suggestions to develop a therapeutic approach aimed at the elimination of CD 4+ OX-40+ lymphocytes are not supported by our findings.

AC133 항원 양성 급성백혈병의 생물학적 특성

김진석,맹호영,정준원,이승태,한지숙,고윤웅,민유홍 大韓血液學會 2002 大韓血液學會誌 Vol.37 No.3

기관지천식 환자에서 흡입성 알레르겐에 대한 피부반응도의 변화양상

이재훈,최소래,홍천수,박중원,김철우,정혜원,정준원 대한알레르기학회 2001 천식 및 알레르기 Vol.21 No.2

Background : Skin prick test has been the primary diagnostic tool used to recognize causative allergens, and there is some evidence that skin reactivity to allergens can be altered by changes in the environment. Objective : We performed this study to evaluate whether there are changes in skin reactivity to inhalant allergens among patients with bronchial asthma. Materials and method : We reviewed the skin prick test results of 384 bronchial asthmatics tested in the early 1990s (June 1992-May 1994) with 52 common inhalant allergens and compared the results of 500 asthmatics tested in the mid 1980s (Jan 1984 - May 1987) with the same kinds of allergens provided by the same manufacturer. Results : Of the 384 subjects tested in the early 1990s, 276 subjects(71.9%) had positive reaction to one or more inhalant allergens and the positive rate was not different from that of the mid 1980s(74%). But, skin reactivity to pollens was significantly increased. In individual allergens, house dust mite(D. farinae and D. pteronyssinus) was the most reactive allergen (52.3%, 43.2% respectively) in the early 1990s. Significant differences in skin reactivity to ragweed (10.0% vs 22.1%, p$lt;0.05) and cockroach (12.7% vs 25.0%, p$lt;0.05) were found when the results of the 1980s and the 1990s were compared. Conclusion : House dust mite was the most important allergen in asthmatics from the mid 1980s to the early 1990s. There was increased sensitization to cockroach and ragweed allergen in the early 1990s. Further prospective immunologic and environmental studies are recommended for proper interpretation of the changes in skin reactivity.