• Journal of Genetic Medicine
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• 제6권1호
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• pp.91-94
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• 2009

저자들은 출생당시 특이소견 없었고, 유전질환의 가족력이 없는 20세 남자 환자에서 우측하지에 골전이가 동반된 횡문근 육종을 발견하고, 저신장, 탈모, 백내장, 치열이상, 골다공증, 갑상선기능저하증, 작은 손과 발, 사지는 가늘고 체간이 두터우며, 신체에 비해 큰 머리등 특이한 외모를 가진 워너증후군으로 진단한 1례를 경험하였기에 문헌 고찰과 함께 보고하고자 한다.

• 동의생리병리학회지
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• 제27권2호
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• pp.153-166
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• 2013

Yang deficiency pattern is a representative syndrome differentiation. This article is a study on to which categories of modern diseases yang deficiency is assigned by reference to modern clinical papers and the meaning of yang deficiency interpreted with a perspective of Korean Medicine and a modern perspective. Yang deficiency, yang qi deficiency, lack of yang qi and yang qi debilitation are the words found in "Nei Ching" and yang qi can be interpreted as something to warm, drive and arouse. Zhangzhongjing considered recovery or loss of Yang as the key to life in "Shanghanlun". Danxi proposed "Yang being liable to hyperactivity, Yin being insufficient" and emphasized pathological ministerial fire of Yang exuberance rather than physiological ministerial fire of Yang deficiency. Zhangjingyue proposed "Yang not being in excess, Yin being often deficient" and understood growth and decline of yin qi are all led by yang qi and put emphasis on true yin in addition to yang qi. Diseases of yang deficiency pattern are related with decline of metabolic level, hypofunction of internal secretion, disorder of immune function, disorder of automatic nerve system, sympathetic nerve inhibition, metabolic disorder of microelements, increase of cGMP, change of microcirculation, low speed of blood stream, kidney malfunction. Diseases related with kidney are sterility, polycystic ovary syndrome, spinal stenosis, edema, renal failure, IgA nephropathy, erectile dysfunction, nephritis, prostatitis, benign prostatic hyperplasia, decrease of adrenal cortical hormone by nephrotic syndrome, myelodysplastic syndrome. Disease related with heart are heart failure, arrhythmia, cardiomyopathy, atherosclerosis heart disease, hypertension, hyperlipidemia, pulmonary heart disease. Diseases related with spleen are irritable bowel syndrome, ulcerative colitis. Diseases related with liver are hypothyroidism, liver cirrhosis ascites, hepatitis B, chronic hepatitis, hepatic diabetes. Diseases related with lung are allergic rhinitis, cough variant asthma, bronchial asthma, pulmonary emphysema. And diabetes mellitus, metabolic syndrome, aplastic anemia, headache, encephalatrophy, Alzheimer's disease are also related with yang deficiency.

• Journal of Trauma and Injury
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• 제33권2호
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• pp.112-118
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• 2020

In trauma patients, coagulopathy and abnormal increases or decreases in cell counts are frequently observed, and are associated with high mortality and morbidity in the acute phase of trauma. Because major trauma is often life-threatening, and hematologic abnormalities are multi-factorial and transient, major blood loss is usually suspected to be the primary cause of these abnormalities, and much time and cost may be spent attempting to identify a focus of hemorrhage that might or might not actually exist. Persistent abnormalities in the complete blood count, however, require clinical suspicion of other hematologic diseases to minimize improper transfusions and to improve outcomes, including mortality. Physicians at trauma centers should be familiar with the clinical characteristics of hematologic diseases and should consider these diseases in trauma patients. In this report, we present cases of two hematologic disorders found in trauma patients: autoimmune hemolytic anemia induced by systemic lupus erythematosus and myelodysplastic syndrome.

• 대한핵의학회지
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• 제36권5호
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• pp.298-305
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• 2002

목적: 혈액 종양 질환을 가진 한자들에서 과립구에 대한 단일클론항체를 이용한 골수 스캔을 시행하여 혈액 종양 질환의 종류에 따라서 말초 골수의 분포를 등급화하고 중심성 골수의 정량적 섭취율을 구하였으며 또한 이들 상호간의 관련성을 조사하였다. 대상 및 방법: 혈액 종양 환자 중에서 골수 조직검사를 시행하여 진단이 확진되고 Tc-99m antigranulocyte monoclonal mouse antibody BW 250/183를 이용한 골수 스캔을 시행한 환자 50명을 대상으로하였다. 환자는 4군으로서 급성 골수성 백혈병 환자 11명, 급성 임파구성 백혈병 환자 12명, 임파선종 환자 15명, 골수이형성증후군 환자 12명이었다. 골수 스캔의 분석은 말초 골수 분포는 골수 확장 소견에 따라 4개의 등급으로 분류하였고, 중심성 골수 기능은 장골과 천골에 관심영역을 그려 섭취율을 계산하였다. 결과: 말초 골수 확장의 정도는 전체 환자 50명 중에서 1등급에 4명, 2등급에 27명, 3등급에 15명, 4등급에 4명 이었다. 말초 골수 확장의 정도는 골수이형성증후군과 임파구성 백혈병에서 3, 4등급이 58% (7/12)로 심하였고 임파종에서는 1, 2등급이 93% (14/15)로 가장 경하였으며 질환별로 특이적인 분포는 보이지 않았다. 중심성 골수의 섭취율은 4가지 질환 중 골수이형성증후군에서 $8.5{\pm}4.0$으로 가장 높았고, 급성 임파구성 백혈병에서 $5.9{\pm}3.6$으로 가장 낮았으나 4군 간에 통계적으로 유의한 차이는 없었다(p>0.05). 말초 골수 화장 정도에 따른 중심 골수의 정량화는 4가지 등급간에는 섭취율에 있어서 통계적으로도 유의한 차이가 있었으나(p=0.003), 개개의 환자에서 말초 골수 확장 정도와 중심 골수의 정량값의 상관관계는 거의 없었다(r=0.05). 결론: 혈액 종양 질환에서 골수 스캔은 말초 골수의 분포와 중심 골수의 기능에 있어서 질병 특이적인 소견은 보이지 않았으며, 골수의 변화는 말초 골수와 중심 골수에 서로 상관 관계없이 변화를 초래하므로 한 가지 방법으로 보다는 두 가지 방법으로 평가할 필요가 있다.

• Clinical and Experimental Pediatrics
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• 제45권3호
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• pp.370-375
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• 2002

목 적 : 소아의 골수이형성 증후군은 드문 질환군으로 예후가 매우 불량하며 화학요법으로는 완치가 어렵다. 유일한 완치요법으로서 조혈모세포이식이 시행되고 있으나 소아의 경우 증례가 적어 이에 대한 체계적 결과 분석이 빈약한 실정이다. 저자들은 골수이형성증후군에서 조혈모세포이식을 시행한 증례들의 단기간의 결과와 이식합병증들을 분석하고자 하였다. 방 법 : 1995년 11월부터 2001년 1월까지 가톨릭대학교 성모병원 소아과에서 골수이형성 증후군으로 조혈모세포이식을 시행 받은 10명의 환아를 대상으로 하였다. 대상질환은 CMMoL 5례, RAEB 3례, RAEBt 2례이었고, 이식형태는 HLA-일치 형제간 골수이식 4례, 비혈연간 골수이식이 4례, 제대혈 조혈모세포이식이 1례, 가족간 HLA-부분일치 조혈모세포이식이 1례이었다. 전처치로는 BuCy 5례, TBI＋BuCy 2례, BuCy＋ATG, TBI＋Cy 및 TBI＋Melphalan이 각각 1례에서 사용되었다. 결 과 : 1) 10명 모두 생착(100%)되었으며 현재 8명(80%)이 무병생존(3-65개월, 정중 추적기간 11개월) 중이다. 2) 이식전처치로 인한 합병증으로 VOD가 3례에서 관찰되었으나 사망한 예는 없었다. 3) II-III도의 급성 이식편대 숙주병은 5례(50%)에서 발생하였으며 II가 4례, III가 1례이었다. 급성 이식 편대 숙주병과 관련된 사망은 없었다. 4) 전체 환아 10례 중 3례에서 이식 후 재발되었으나 1례는 화학요법 후 조혈모세포구제술에 의하여 현재 무병생존 중이며 2례는 사망하였다. 결 론: 소아 골수이형성 증후군에서 조혈모세포이식은 질환을 완치시킬 수 있는 우수한 결과를 보여주고 있으나 아직 증례가 적고 추적기간이 짧아 향후 더 많은 연구가 필요하다.

• The Korean journal of internal medicine
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• 제33권6호
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• pp.1194-1202
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• 2018

Background/Aims: This study evaluated the role of hypomethylating agents (HMA) compared to best supportive care (BSC) for patients with high or very-high (H/VH) risk myelodysplastic syndrome (MDS) according to the Revised International Prognostic Scoring System. Methods: A total of 279 H/VH risk MDS patients registered in the Korean MDS Working Party database were retrospectively analyzed. Results: HMA therapy was administered to 205 patients (73.5%), including 31 patients (11.1%) who then received allogeneic hematopoietic cell transplantation (allo-HCT), while 74 patients (26.5%) received BSC or allo-HCT without HMA. The 3-year overall survival (OS) rates were $53.1%{\pm}10.7%$ for allo-HCT with HMA, $75%{\pm}21.7%$ for allo-HCT without HMA, $17.3%{\pm}3.6%$ for HMA, and $20.8%{\pm}6.9%$ for BSC groups (p < 0.001). In the multivariate analysis, only allo-HCT was related with favorable OS (hazard ratio [HR], 0.356; p = 0.002), while very poor cytogenetic risk (HR, 5.696; p = 0.042), age ${\geq}65years$ (HR, 1.578; p = 0.022), Eastern Cooperative Oncology Group performance status (ECOG PS) 2 to 4 (HR, 2.837; p < 0.001), and transformation to acute myeloid leukemia (AML) (HR, 1.901; p = 0.001) all had an adverse effect on OS. Conclusions: For the H/VH risk group, very poor cytogenetic risk, age ${\geq}65years$, ECOG PS 2 to 4, and AML transformation were poor prognostic factors. HMA showed no benefit in terms of OS when compared to BSC. Allo-HCT was the only factor predicting a favorable long-term outcome. The use of HMA therapy did not seem to have an adverse effect on the transplantation outcomes. However, the conclusion of this study should be carefully interpreted and proven by large scale research in the future.

• Asian Pacific Journal of Cancer Prevention
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• 제16권15호
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• pp.6627-6632
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• 2015

Background: We conducted a study exploring the clinical safety and efficacy of decitabine in patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS), combined with a complex karyotype. Materials and Methods: From April 2009 to September 2013, a total of 35 patients with AML/MDS combined with a complex karyotype diagnosed in the First Affiliated Hospital of Soochow University were included for retrospective analysis. All patients were treated with decitabine alone ($20mg/m^2$ daily for 5 days) or combination AAG chemotherapy (Acla 20mg qod*4d, Ara-C $10mg/m^2$ q12h*7d, G-CSF $300{\mu}g$ qd, the dose of G-CSF adjusted to the amount in blood routinely). Results: In 35 patients, 15 exhibited a complete response (CR), and 6 a partial response (PR), the overall response rate (CR+PR) being 60% (21 of 35). Median disease-free survival was 18 months and overall survival was 14 months. In the 15 MDS patients with a complex karyotype, the CR rate was 53.3% (8 of 15); in 20 AML patients with complex karyotype, the overall response rate was 65% (13 of 20). The response rate of decitabine alone (22 cases) was 56.5% (13 of 22), while in the combination chemotherapy group (13 cases), the effective rate was 61.5% (8 of 13)(P>0.05). There are 15 patients with chromosome 7 aberration, after treatment with decitabine, 7 CR, 3 PR, overall response rate was 66.7% (10 of 15). Of 18 patients with 3 to 5 kinds of chromosomal abnormalities, 66.7% demonstrated a response; of 17 with more than 5 chromosomal abnormalities, 52.9% had a response. In the total of 35 patients, with one course (23 patients) and ${\geq}$two courses (12 patients), the overall response rate was 40.9% and 92.3% (P<0.05). Grade III to IV hematological toxicity was observed in 27 cases (75%). Grade III to IV infections were clinically documented in 7 (20%). Grades I to II non-hematological toxicity were infections (18 patients), haematuria (2 patients), and bleeding (3 patients). With follow-up until September 2013, 7 patients were surviving, 18 had died and 10 were lost to follow-up. In the 6 cases who underwent allogeneic hematopoietic stem cell transplantation (HSCT) all were still relapse-free survivors. Conclusions: Decitabine alone or combination with AAG can improve outcome of AML/MDS with a complex karyotype, there being no significant difference decitabine in inducing remission rates in patients with different karyotype. Increasing the number of courses can improve efficiency. This approach with fewer treatment side effects in patients with a better tolerance should be employed in order to create an improved subsequent chance for HSCT.

• Journal of Chest Surgery
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• 제51권5호
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• pp.350-355
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• 2018

Background: The complication rate of fungal disease is higher among patients with hematological malignancies. We investigated the clinicobacteriological outcomes of resected pulmonary fungal infections complicating hematological malignancies. Methods: Between 2001 and 2017, 21 patients with pulmonary fungal infections complicating hematological malignancies underwent resection, and their clinical records and survival were retrospectively reviewed. Results: The median age of the patients was 47 years, and 13 were male. The histological diagnoses were pulmonary aspergillosis (19 cases), mucormycosis (1 case), and cryptococcosis (1 case). The indications for surgery were resistance to antifungal therapy and the necessity of surgery before hematopoietic stem cell transplantation in 13 and 8 cases, respectively. The diagnoses of the hematological malignancies were acute myelogenous leukemia (10 cases), acute lymphocytic leukemia (5 cases), myelodysplastic syndrome (3 cases), and chronic myelogenous leukemia, malignant lymphoma, and extramedullary plasmacytoma (1 case each). The surgical procedures were partial resection (11 cases), segmentectomy (5 cases), lobectomy (4 cases), and cavernostomy (1 case). The size of the lesions was 0.9-8.5 cm. Fourteen cases had cavitation. There were no surgical-related deaths or fungal progression. Conclusion: Pulmonary fungal infections are resistant to treatments for hematological malignancies. Since the treatment of the underlying disease is extended and these infections often recur and are exacerbated, surgery should be considered when possible.

• Advances in pediatric surgery
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• 제10권1호
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• pp.31-34
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• 2004

The laparoscopic splenectomy (LS) became popular over the last 10 years. The advantage of LS over open splenectomy (OS) includes short hospital stay, improved cosmesis, less development of postoperative intestinal ileus, and less analgesics required. The purpose of this study is to evaluate the outcome of LS at Asan Medical Center from January 1999 to January 2003. The records of 14 consequent children who underwent splenectomy were reviewed retrospectively. Patients characteristics, morbidity, mortality, operative time, blood loss, and hospital stay were analyzed., Seven patients age 5 to 15 years underwent LS under the indications: idiopathic thrombocytic purpura (ITP, n=3), hereditary spherocytosis (n=3), and myelodysplastic syndrome (n=l). Seven patients, age 7 to 16 years, underwent OS during the same period for ITP (n=7). Median operative time was 120 mInutes (80 to 170 mins.) in OS, and 270 minutes (110 to 480 mins,) in LS (p<0.05). Median length of hospital stay was 6 days (3 to 8) in OS, and 4 days (3 to 6) in LS (p>0.05). Median splenic length was 12.0cm (9.2 to 18.0) in OS, 14.0 cm (10.0 to 19.5) (p>0.05). Accessory spleens were identified in 3 of 7 LS and 1 of 7 OS cases. In the LS group, there was no conversion to open surgery. Two patients in LS required blood transfusion postoperatively. LS in children can be performed as effectively and safely as OS.

• Journal of Chest Surgery
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• 제49권3호
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• pp.195-198
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• 2016

We report a 72-year-old male with known myelodysplastic syndrome who presented to the emergency department with a 7-day history of fever and dyspnea. Echocardiography revealed a round echogenic mass $13{\times}16mm$ in size attached to the atrial side of the tricuspid valve. Considering the high risk of infective endocarditis in the patient with a low absolute neutrophil count ($130/mm^3$), emergency surgery was performed. Intraoperatively, a single gelatinous neoplasm was resected, and subsequent reconstruction of the involved leaflet was accomplished using autologous pericardium. The tumor was pathologically confirmed as papillary fibroelastoma with no evidence of infective endocarditis. Papillary fibroelastoma is a rare cardiac neoplasm that occurs in either the mitral or aortic valves. Interestingly, a few cases of tricuspid valve papillary fibroelastoma have been reported so far. Similar echocardiographic findings between vegetation and tricuspid valve neoplasm make it difficult to distinguish these two disease entities.