성인 급성골수성백혈병 치료 전략의 새로운 변화

정준원,민유홍 대한의사협회 2023 대한의사협회지 Vol.66 No.4

Background: Acute myeloid leukemia (AML) is a representative blood cancer, accounting for most adult leukemia cases in Korea. Until recently, intensive chemotherapy and hematopoietic stem cell transplantation were the only curative treatment options for AML. However, the recent introduction of new drugs is bringing about changes in the strategic paradigm for the treatment of AML. Current Concepts: Along with the clinical eligibility for receiving intensive treatment and hematopoietic stem cell transplantation, the most critical determinants in treating AML are precise classification and risk stratification based on cytogenetic and molecular information. The recently revised World Health Organization classification, newly proposed International Consensus Classification, and the latest version of the European LeukemiaNet risk stratification reflect the importance of cytogenetic and molecular information. Although there have been no significant changes for a long time in the landscape of AML, especially in the field of treatment, the treatment paradigm has started to evolve with the introduction of new drugs. This evolution is led by FLT3 inhibitors, Bcl-2 inhibitors, isocitrate dehydrogenase inhibitors, target agents against CD33 antigens, and liposomal formulations of chemotherapeutics. Discussion and Conclusion: Successful initial treatment to induce complete remission followed by postremission treatment to remove residual disease can lead to the achievement of long-term survival and cure goals in AML. We hope that new drugs will markedly improve the treatment outcomes for patients with AML.

노인 빈혈의 진단과 치료

정준원 대한내과학회 2021 대한내과학회 춘계학술대회 Vol.2021 No.-

A Substrate Mimetic Approach for Influenza Neuraminidase Inhibitors

정준원,Jae Kuk Kim,우병영,송복주,하덕찬,Zaesung No 대한화학회 2004 Bulletin of the Korean Chemical Society Vol.25 No.10

골수형성이상증후군에서의 임상진료지침

정준원,국훈,방수미,이제환,주영돈,김인호,김형준,박찬정,박현진,안진석,윤성수,원종호,이홍기,정철원,조덕연,조빈,한경자,민유홍,김선희 대한혈액학회 2007 Blood Research Vol.42 No.2

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.

급성골수구성백혈병의 최신지견

정준원 대한내과학회 2018 대한내과학회 추계학술대회 Vol.2018 No.2

효소 면역 측정법에서 음성인 AIDS 뇌병증 환자의 부검 1예

정준원,류지소,정진원,강수진,박이내,조영걸,강신관,우준희,김양수 대한감염학회 2001 감염 Vol.33 No.3

The diagnosis of Human Immunodeficiency Virus (HIV) infection is established serologically. The screening test of HIV infection is enzyme-linked immunosorbent assay (ELISA) and its sensitivity ranges from 93 to 100%. The false negative results of ELISA can occur during primary HIV infection and late stage AIDS patients. Patients who are clinically suspicious HIV infection but repeatedly negative ELISA should be evaluated with serologic assays and method for directly detecting viral antigen and nucleic acid. We report an autopsy case of 32 year-old man who was repeatedly negative in ELISA, and was diagnosed by polymerase chain reaction (PCR) and p24 antigenemia assay. We observed the microglial nodule in the brain, which was the pathologic basis for the clinical HIV encephalopathy. (Korean J Infect Dis 33:227∼231, 2001)

급성골수성백혈병에서 Notch 발현의 의미

정준원,김진석,이혜원,윤설희,김수정,민유홍 대한혈액학회 2008 Blood Research Vol.43 No.1

Background: Notch is a gene family encoding receptors to transduce intercellular signals involved in cell-fate determination. Although several lines of evidence indicate that abnormal Notch signaling may contribute to neoplastic transformation, little is known regarding the role of Notch in the pathogenesis of leukemia. Methods: To explore the functional significance of Notch1 in acute myeloid leukemia (AML), the expression of Notch1 and its association with survivin and p27Kip1 expression was examined in 50 patients with de novo AML. Results: Notch1 transcripts were expressed in 40 (80%) cases with a variable degree of expression, and the fraction of AML cells in the G0/G1 phase was higher in Notch1-positive cases than in Notch1-negative cases. Survivin was shown to be present in 38 (76.0%) cases, and Notch1 expression highly correlated with survivin mRNA expression (r=0.7170, P<0.001). p27Kip1 was present in 40 (80.0%) cases of AML and p27Kip1 expression was significantly associated with Notch1 expression (r=0.8770, P<0.001). Except for one case, the simultaneous expression of survivin and p27Kip1 was not seen in all cases that were negative for Notch1 expression. There were no differences in clinical outcomes according to Notch1 expression. Conclusion: Notch1 expression was a frequent event and has functional significance in the alteration of the cell cycle in AML cells. Notch1 expression was also significantly associated with survivin and p27kip1 expression in AML cells. To evaluate the clinical significanceand functional role of Notch1 expression in the aberrant regulation of survivin and p27Kip1 expression in de novo AML, a further study with a larger number of patients is necessary.

경쟁학습 신경회로망을 이용한 텍스트종속 화자인식 ( Text-Dependent Speaker Verification Using Competitive Learning Neural Network )

정준원,조성원 대한전자공학회 1996 대한전자공학회 학술대회 Vol.1996 No.1

Palisading Vessel의 하단과 Z-line의 불일치 비율 및 검사자 간 일치도

정준원,이진혁,최기돈,송호준,김병규,최귀숙,정훈용,김진호 대한소화기내시경학회 2007 Clinical Endoscopy Vol.34 No.5

Background/Aims: The Barrett's esophagus is confirmed by performing a biopsy when the gastroesophageal junction (GEJ) and Z-line do not coincide. In Japan, the GEJ is at the distal end of the palisading vessel while Western countries define it as the proximal tip of the gastric fold. However, there is little data on the prevalence of an endoscopic Barrett's esophagus and the inter-observer variation. Methods: Four experienced endoscopists reviewed the endoscopic still images of 111 consecutive patients. The level of inter-observer agreement was expressed as a kappa value. Results: The average percentage of patients with an endoscopically confirmed esophagus was 34.2%. The level of inter-observer agreement was substantial (kappa=0.698). Conclusions: The prevalence of an endoscopic confirmed Barrett's esophagus was high, and the inter-observer variation was substantial when the GEJ was defined as the distal end of the palisading vessel. Considering the low incidence of esophageal adenocarcinoma and the risk of hemorrhage from a biopsy, a more specific marker is needed in this high-risk group. (Korean J Gastrointest Endosc 2007;34:239-243) 목적: 최근 서구에서 바렛식도에서 발생하는 식도 선암이 증가하고 있다. 바렛식도를 진단하기 위한 조직검사는 위식도 접합부와 Z-line이 불일치를 보일 경우 시행한다. 내시경검사에서 위식도의 접합부는 서구에서는 위주름의 최근위부로 정의하고 있고, 일본에서는 palisading vessel의 최하단부를 기준으로 하고 있다. 그러나 국내에서 palisading vessel을 기준으로 하였을 때 Z-line과 위식도부 접합부의 내시경검사 불일치비율이 얼마인지에 대하여 잘 알려져 있지 않다. 이에 저자들은 palisading vessel을 기준으로 하여, Z-line과 위식도 접합부의 불일치의 비율과 이에 대한 검사자 간의 일치도에 대하여 연구하였다. 대상 및 방법: 2006년 4월부터 6월까지 내시경 검사를 받기 위해 방문한 111명을 대상으로 Z-line과 palisading vessel의 최하단부의 불일치 여부를 4명의 검사자가 판정하였다. 결과: 환자의 평균나이는 56.2±11.3세(26~82세)였고, 남녀비는 55:56이었다. 내시경 검사에서 역류성 식도염이 있는 경우는 6.3% (7/111명)였다. 각 연구자의 불일치 판정 비율은 각각 25.2%, 33.3%, 35.1%, 43.2%였고 평균값은 34.2±7.3%였다. 이에 대한 kappa 값은 0.698였다. 검사자 사이에서 서로 다른 판정은 주로 위식도 접합부의 점막이 과립상을 보이거나, Z-line 하방의 혈관이 그물상을 보일 때 발생하였다. 결론: Palisading vessel을 기준으로 하였을 때 불일치 판정의 검사자간 일치도는 높았으며, 불일치 비율은 다른 지표를 기준으로 하였던 보고에 비하여 높았다. 우리나라에서 식도 선암의 낮은 유병률과 조직검사의 출혈위험을 고려할 때 고위험군에 좀더 특이적인 지표에 대한 연구가 필요하다.