고위험군의 원발성 전신성 유전분증 1례 보고 및 조혈모세포이식에 대한 문헌고찰

심준,박수정,엄현석,김기영,박은정,강인중,조병식,이안희,한치화 대한조혈모세포이식학회 2001 대한조혈모세포이식학회지 Vol.6 No.2

저자 등은 클론성 형질세포질환과 동반된 젊은 연령의 원발성 전신성 유전분증 환자를 진단하였기에 다발성 골수종과의 감별 진단, 치료 , 예후 및 고용량 항암화학요법과 조혈모세포이식에 관하여 문헌고찰과 함께 보고하는 바이다. Primary systemic amyloidosis (AL) is a rapidly fatal disorder related to plasma cell dyscrasia. Conventional dose of melphalan, which prolongs the duration of survival by about 10 months, does not improve the functions of impaired organs in most cases. The high dose chemotherapy followed by autologous hematopoietic stem cell rescue for AL, in spite of its high treatment-related mortality, is a new approach to achieve high response rate and better survival. We experienced a 35-year old man with AL(involving heart, liver, stomach, kidneys, peripheral nerve, and rectum) who did not respond to the standard schedule of melphalan plus prednisone and had rapidly fatal course with organ failure. Hence, we evaluate its availability by reviewing the recent reports of high dose chemotherapy in AL.

암 환자의 우울과 통증 : 일 예비적 연구

양문정,전양환,한상익,한치화,엄현석 대한신경정신의학회 2000 신경정신의학 Vol.39 No.6

연구목적 : 암 환자에서 우울과 통증의 정도, 우울과 통증에 영향을 줄 수 있는 요인들을 조사하고, 우울과 통증사이에 어떤 연관이 있는지 알아보고자 하였다. 방 법 : 종양내과에 입원한 암 환자 25명(남자 : 10명, 여자 :15명)을 대상으로 하여 내과 병록지 거모, 환자 및 가족과의 면담을 시행하였다. 정신과적 진단은 DSM-IV진단 기준에 의하였으며, 우울의 정도는 Hamilton Rating Scale for Depression(HRSD)로 평가하였고 통증의 유무를 평가한 뒤, 통증이 있는 경우 Brief Pain Inventory(BPI)를 시행하여 통증의 강도(최대, 최소,평균,현재), 통증에 의한 기능 방해 정도, 진통제에 의한 호전정도를 점수로 평가하였다. 결 과 : 전체 환자중 주요 우울증이 32%(8명), 경도 우울증이 16%(4명), 적응장애가 16%(4명)이었다. HRSD점수는 통증의 최대강도, 평균강도, 현재강도, 통증에 의한 기능방해정도와 유의한 상관관계가 있었으나, 통증의 최소강도, 진통제에 의한 호전 정도와는 유의한 상관관계가 없었으며, 이환 기간과 HRSD점수 및 통증 점수 사이에도 유의한 상관관계가 없었다. 성별, 종교, 암의전이여부,병황의 인식 여부에 따라 HRSD점수와 통증 점수에 차이가 없었으나, 현재 미혼 또는 사별 상태에 있는 환자들에서 결혼하여 배우자가 있는 환자들보다 HRSD점수와 통증의 최소강도, 평균강도가 유의하게 높았다. 결 론 : 암환자에게서는 우울증과 통증이 있는 경우가 많았다. 우울증과 통증간에 밀접한 상관관계가 있음을 알수 있었다. 본 연구의 결과는 암 환자에서 우울증과 통증에 대한 보다 적극적인 평가와 개입이 이루어져야 함을 시사하는 것이다. Objective : This study was designed to evaluate how much depression and pain symptoms could be shown, what kink of factors affect them, and whether the correlation between them could be or not in patients with cancer. Methods : The subjects were composed of 25 patients with cancer who admitted a the department of oncology(male:10, female :15). We reviewed the medical record and interviewed patients and their family. A psychiatric diagnosis was made according to the criteria of the DSM-IV, and depressive symptoms were evaluated by Hamilton Rating Scale for Depression(HRSD). The intensity of pain(maximal, minimal, mean, present), disability due to pain, te effects of analgesics were measured by Brief Pain Inventory(BPI). Results : 32% of patients had major depressive disorders, 16% of patients had depressive disorders, NOS and 16% of the patients had adjustment disorders. The score of HRSD was significantly correlated wit the maximal intensity, mean intensity and present intensity of pain and disability due to pain, but not with minimal intensity and the effects of analgesics. Depression and pain were not correlated with duration of illness. Scores of depression and pain did not differ in sex, religion, metastasis, and the knowledge of illness. The widowed or unmarried patients showed significantly higher scores than patients living with the spouse in HRSD, minimal intensity and mean intensity of pain. Conclusion : In patients with cancer, depression and pain were highly prevalant. The relationship between depression and pain was shown in patients with cancer. These results suggest that more active evaluation and intervention of depression and pain should be carried out in patients with cancer.

Bortezomib, thalidomide, dexamethasone induction therapy followed by melphalan, prednisolone, thalidomide consolidation therapy as a first line of treatment for patients with multiple myeloma who are non-transplant candidates: results of the Korean Multi

Eom, Hyeon-Seok,Kim, Yeo-Kyeoung,Chung, Joo-Seop,Kim, Kihyun,Kim, Hyo Jung,Kim, Ho Young,Jin, Jong-Youl,Do, Young-Rok,Oh, Suk-Joong,Suh, Cheolwon,Seong, Chu-Myong,Kim, Chul Soo,Lee, Dong Soon,Lee, Jae Springer-Verlag 2010 Annals of hematology Vol.89 No.5

Absence of oncogenic AKT1 E17K mutation in prostate, esophageal, laryngeal and urothelial carcinomas, hepatoblastomas, gastrointestinal stromal tumors and malignant meningiomas.

Eom, Hyeon Seok,Kim, Min Sung,Hur, Soo Young,Yoo, Nam Jin,Lee, Sug Hyung Acta Oncologica 2009 Acta Oncologica Vol.48 No.7

PML/RAR-α 유전자 동형의 임상적 의의가 있는가?

엄현석 ( Hyeon Seok Eom ) 대한내과학회 2008 대한내과학회지 Vol.75 No.4

Acute promyelocytic leukemia (APL) is characterized by a specific t (15;17) translocation which produce a PML/RAR-α fusion messenger RNA and by effectiveness of all-trans retinoic acid (ATRA) differentiation therapy. Breakpoints within PML intron 3 (bcr 3) produce a short PML/RAR-α isoform (S-isoform), whereas breakpoints within PML intron 6 (bcr 1) result in a longer form (L-isoform). Additionally, breakpoints within PML exon 6 (bcr 2) make a variable length transcript (V-isoform) in a small number of patients. The influence of breakpoint site on patient outcome remains controversial. Previous reports showed that patients with S-isoform have an increased incidence of clinical relapse and shorter survival compared to those with L-isoform. Others reported no difference in DFS between these patients groups. In this issue, Lee et al. reported that there were 58 L-isoform (62.1%), 32 S-isoform (34.0%), 4 V-isoform (4.3%) and, no significant prognostic factor for EFS from induction therapy using anthracycline plus ATRA among 94 patients with APL. They concluded pretreatment clinical characteristics and treatment outcomes were not significantly different according to PML/RAR-α isoform types in this induction group. Recently, it was reported that FLT3/ITD mutation was frequently associated with S-isoform and with the M3v form of leukemia and CNS relapse in APL was mostly related to S-isoform. With previous studies including this article, outcomes of different types of PML/RAR-α isoforms are not conclusive. Future researches need to be focused not only on clinical outcomes of different types of PML/RAR-α isoforms, but also clinical relevance of PML/RARA-α mRNA isoforms with other prognostic factors and particular clinical characteristics. (Korean J Med 75:409-411, 2008)

정상 위 분문부 점막에서 발견된 이소성 골 형성

엄석현 ( Seok Hyeon Eom ),박창환 ( Chang Hwan Park ),정덕원 ( Duk Won Chung ),이상혁 ( Sang Hyeok Lee ),서지영 ( Ji Young Seo ),김영성 ( Yeong Sung Kim ),곽동협 ( Dong Hyup Kwak ),김정희 ( Jung Hee Kim ) 영남대학교 의과대학 2016 Yeungnam University Journal of Medicine Vol.33 No.2

Heterotopic bone formation in the gastrointestinal tract is a rare phenomenon. Most reported cases were associated with benign and malignant neoplasms, except for a case in which heterotopic bone formation was found in a patient with Barrett`s esophagus. The exact pathogenesis of the disease has not yet been established. However, most heterotopic bones found in the gastrointestinal tract were associated with mucinproducing tumors of the appendix, colon, and rectum. Inflammation may also play a role in osseous metaplasia in a case with bone formation at the base of an ulcer in Barrett`s esophagus. Here, we report on a patient with heterotopic bone formation in normal gastric cardiac mucosa. A 50-year-old female visited our hospital for a routine health examination. She had no gastrointestinal symptoms, and her physical examination, blood test, X-ray, urine, and stool examination results were normal. A 0.3 cm sized polypoid lesion located just below the squamocolumnar junction was observed on upper gastrointestinal endoscopy. A piece of biopsy was taken. Histologically, a lamella bone trabecula and chronic inflammatory cells were observed in the gastric cardiac mucosa. The follow-up endoscopy performed one month later showed no residual lesion.

Concurrent chemoradiotherapy followed by l-asparaginase-containing chemotherapy, VIDL, for localized nasal extranodal NK/T cell lymphoma: CISL08-01 phase II study

Kim, Seok Jin,Yang, Deok-Hwan,Kim, Jin Seok,Kwak, Jae-Yong,Eom, Hyeon-Seok,Hong, Dae Sik,Won, Jong Ho,Lee, Jae Hoon,Yoon, Dok Hyun,Cho, Jaeho,Nam, Taek-Keun,Lee, Sang-wook,Ahn, Yong Chan,Suh, Cheolwon Springer-Verlag 2014 Annals of hematology Vol.93 No.11

Multicenter analysis of treatment outcomes in adult patients with lymphoblastic lymphoma who received hyper-CVAD induction followed by hematopoietic stem cell transplantation.

Jeong, Seong Hyun,Moon, Joon Ho,Kim, Jin Seok,Yang, Deok-Hwan,Park, Yong,Cho, Seok Goo,Kwak, Jae-Yong,Eom, Hyeon Seok,Won, Jong Ho,Hong, Jun Shik,Oh, Sung Yong,Lee, Ho Sup,Kim, Seok Jin Springer International 2015 Annals of hematology Vol.94 No.4

<P>The hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD) regimen has been widely used for lymphoblastic lymphoma (LBL) as a primary treatment. However, there is few data about its treatment outcome in Asian patients. Thus, we conducted this study to evaluate the efficacy of hyper-CVAD induction and stem cell transplantation (SCT) consolidation in LBL patients. The treatment responses of 49 patients treated with the hyper-CVAD regimen were retrospectively analyzed in 13 institutions. Given 24 patients who responded to hyper-CVAD underwent consolidation treatment with SCT, overall survival (OS) and progression-free survival (PFS) of patients who received SCT were compared with patients who did not. The overall response rate was 79 %: 73 % (36/49) complete responses, 6 % (3/49) partial responses, and 4 % (2/49) induction deaths. The major limitation for the delivery of the planned hyper-CVAD cycles was hematological toxicity. Among 39 responders, 24 patients underwent autologous (n?=?16) and allogeneic SCT (n?=?8) consolidation. Their 3-year OS and PFS rates were 76 and 78 %, respectively, and there was no difference in survival outcomes between autologous and allogeneic SCT. However, 15 patients without SCT consolidation showed poorer PFS even though they all achieved complete response. Thus, only seven patients maintained their response at the time of analysis. In conclusion, the hyper-CVAD regimen is effective for remission induction in LBL, and SCT consolidation after hyper-CVAD induction produced better clinical outcomes than did continuation of hyper-CVAD.</P>

골수이식 후 발생한 이차성 악성 종양

엄현석,김희제,민창기,최정현,이종욱,한치화,최일봉,민우성,신완식,김춘추,한경자,강창석,김원일,박재후,김동집 대한조혈모세포이식학회 1997 대한조혈모세포이식학회지 Vol.2 No.1

연구배경: 골수이식은 혈액암, 고형암, 기타 질환들에서 근본적인 치료법으로 자리를 잡아가며 널리 시행되고 있으나 생존율이 향상되면서 전처치 방사선이나 고용량 화학요법으로 인한 이차성 악성종양의 발생이 중요한 후기 합병증으로 보고 되고 있어, 국내에서의 골수이식 환자에서 이차성 악성 종양의 빈도와 연관된 위험 인자에 대하여 알아보고자 후향적 조사를 시행하였다. 방법: 가톨릭 골수이식센터에서 1983년부터 1997년 4월까지 시행한 골수이식 534예 중에서발생된 이차성 악성 종양을 후향적으로 조사하였다. 결과: 골수이식 534예 중에서 유방암의 자가 골수이식 후 위선암, 급성 골수성백혈병의 자가골수이식 및 말초 조혈모세포이식 후 갑상선 유듀상암, 골수 이형성 증후군의 동종 골수이식 후 구강 편평세포암, 그리고 재생불량성빈혈의 동종 골수이식 후 EB 바이러스 연관성 림프종 등 4예가 골수 이식 후 각각 27, 56, 9, 3개월에서 발생하였고, 2예에서 전신 방사선 조사를 받았다. 결론: 골수이식은 암이나 기타 난치성 질환 등을 근본적으로 치료할 수 있는 적극적인 치료법이지만 이식 후 생존율을 저하시키는 후기 합병증들을 가지고 있다. 이차성 악성 종양도 흔하지 않으나 피할수 없는 합병증들의 하나이다. 따라서 암전구 증상발견 및 암조기 발견이 중요하며, 암유발 요인을 적극적으로 피하는 것이 중요할 것으로 생각된다. Bone marrow transplantation(BMT) is a widely accepted therapeutic modality for hematologic malignancies, chemo-sensitive solid tumors, and other life-threatening hematologic diseases. However, with the improvement in survival, post-BMT neoplasms are emerging as a serious late complication in this group of survivors. Secondary neoplasms are a known complication of chemotherapy and irradiation used to treat Hodgkin's disease and non-Hodgkin's lymphoma and associated with immunosuppression after solid organ transplantation. The records of 534 consecutive cases of allogeneic, syngeneic and autologous BMT were reviewed of determine the incidence of secondary malignancies among whom received BMT for a variety of hematologic and non-hematologic disorders in Catholic Bone Marrow Transplantation Center since March 1983. Four cases of secondary maligancies were developed. Ebstein-Barr virus associated lymphoproliferative disease in stomach, squamous cell carcinoma in buccal cavity, adenocarcinoma in stomach, papillary cancer in thyroid gland were developed at 3, 9, 27, and 56 months post BMT, respectively. Here we firstly report 4 cases of secondary malignancy after BMT in Korea with review of literatures.

Regulation of Acetylation of Histone Deacetylase 2 by p300/CBP-Associated Factor/Histone Deacetylase 5 in the Development of Cardiac Hypertrophy

Eom, Gwang Hyeon,Nam, Yoon Seok,Oh, Jae Gyun,Choe, Nakwon,Min, Hyun-Ki,Yoo, Eun-Kyung,Kang, Gaeun,Nguyen, Vu Hong,Min, Jung-Joon,Kim, Jong-Keun,Lee, In-Kyu,Bassel-Duby, Rhonda,Olson, Eric N.,Park, Woo Grune & Stratton 2014 Circulation research Vol.114 No.7

<P><B><U>Rationale:</U></B></P><P>Histone deacetylases (HDACs) are closely involved in cardiac reprogramming. Although the functional roles of class I and class IIa HDACs are well established, the significance of interclass crosstalk in the development of cardiac hypertrophy remains unclear.</P><P><B><U>Objective:</U></B></P><P>Recently, we suggested that casein kinase 2α1–dependent phosphorylation of HDAC2 leads to enzymatic activation, which in turn induces cardiac hypertrophy. Here we report an alternative post-translational activation mechanism of HDAC2 that involves acetylation of HDAC2 mediated by p300/CBP-associated factor/HDAC5.</P><P><B><U>Methods and Results:</U></B></P><P>Hdac2 was acetylated in response to hypertrophic stresses in both cardiomyocytes and a mouse model. Acetylation was reduced by a histone acetyltransferase inhibitor but was increased by a nonspecific HDAC inhibitor. The enzymatic activity of Hdac2 was positively correlated with its acetylation status. p300/CBP-associated factor bound to Hdac2 and induced acetylation. The HDAC2 K75 residue was responsible for hypertrophic stress–induced acetylation. The acetylation-resistant Hdac2 K75R showed a significant decrease in phosphorylation on S394, which led to the loss of intrinsic activity. Hdac5, one of class IIa HDACs, directly deacetylated Hdac2. Acetylation of Hdac2 was increased in Hdac5-null mice. When an acetylation-mimicking mutant of Hdac2 was infected into cardiomyocytes, the antihypertrophic effect of either nuclear tethering of Hdac5 with leptomycin B or Hdac5 overexpression was reduced.</P><P><B><U>Conclusions:</U></B></P><P>Taken together, our results suggest a novel mechanism by which the balance of HDAC2 acetylation is regulated by p300/CBP-associated factor and HDAC5 in the development of cardiac hypertrophy.</P>