• Journal of Genetic Medicine
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• v.6 no.1
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• pp.91-94
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• 2009

Werner syndrome (WRN), or adult progeria, is a very rare, autosomal recessive disorder characterized by the appearance of accelerated aging, including cataracts, gray hair, skin atrophy, and atherosclerosis. This syndrome is caused by mutations in the WRN gene and had a high risk of a spectrum of rare neoplasms including: i) non-epithelial malignant or pre-malignant tumors/conditions, osteosarcomas and soft tissue sarcomas, malignant melanomas, myeloid leukemia and myelodysplastic syndrome; ii) an epithelial neoplasm, thyroid carcinoma, and iii) meningiomas. Recently, authors experienced a case of Werner syndrome complicated by bone metastasis of rhabdomyosarcoma in a 20-year old Korean man. The patient revealed a painful mass on his right knee and progeroid features, short stature, scalp alopecia, abnormal dentition, craniofacial disproportion, hypothyroidsm, cataracts and osteoporosis. The onset of symptoms of Werner syndrome generally precedes any later symptoms of associated conditions, such as malignant tumor. Therefore, early recognition of Werner syndrome is important to assist identification of malignant tumors at an early stage in this patient group.

• Journal of Physiology & Pathology in Korean Medicine
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• v.27 no.2
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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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• v.33 no.2
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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.

• The Korean Journal of Nuclear Medicine
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• v.36 no.5
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• pp.298-305
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• 2002

Purpose: Bone marrow scintigraphy has been used to evaluate the status of bone marrow in various hematologic disorders. We have analyzed the peripheral distribution pattern and central uptake ratio of bone marrow using anti-NCA-95 monoclonal antibody and the their correlation in patients with various hematologic malignancy. Materials and Methods: Bone marrow immunoscintigraphy was performed using Tc-99m anti-granulocyte monoclonal mouse antibody BW 250/183. Fifty patients were classified into four groups; 11 with acute myelogenous leukemia, 12 with acute lymphocytic leukemia, 15 with lymphoma and 12 with myelodysplastic syndrome. The extension of peripheral bone marrow was categorized into four grades: I, II, III and IV. The activity of central bene marrow was expressed as sacroiliac uptake ratio. Results: The patient's number was 4 in grade I, 27 in grade II, 15 in grade III and 4 in grade IV according to extension of peripheral bone marrow. The extension of peripheral bone marrow was marked (58% in grade III and IV) in myelodysplastic syndrome and acute lymphocytic leukemia and mild (93% in grade I and II) in lymphoma. Sacroiliac uptake ratio was highest ($8.5{\pm}4.0$) in myelodysplastic syndrome and lowest ($5.9{\pm}3.6$) in acute myelogenous leukemia, but not significantly different among four patient groups (p>0.05). Sacroiliac uptake ratio of whole patients was significantly different among four grades (p=0.003), but there was not correlated between grade of peripheral bone marrow and sacroiliac uptake ratio (r=0.05). Conclusion: The pattern of peripheral bone marrow extension and activity of central hemopoietic marrow were not specific to the disease entities. Response of hemopoietic bone marrow may be evaluated on both peripheral and central bone marrow in patients with hematologic malignancy.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.370-375
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• 2002

Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods : Ten children with MDS(CMMoL 5, RAEB 3, RAEBt 2) underwent HSCT(HLA-matched sibling transplantation 4, HLA-matched unrelated transplantation 4, cord blood transplantation 1, HLA-mismatched familial transplantation 1) between November 1995 and January 2001 at St. Mary's Hospital. Median follow-up duration was 11 months. Results : Engraftment was successful in all cases and 8 patients are alive without disease. Three cases of VOD were observed and improved without complication. Four cases of grade II and 1 case of grade III acute GVHD were observed and well controlled with treatment. Three patients relapsed after transplantation. One patient is alive without disease after cytoreduction with allogenic stem cell rescue and 2 patients died of relapse. Conclusion : HSCT is a curative strategy of MDS and the survival rate is relatively higher than that of adults. But there is an obvious need for more studies because of the small number of patients and the short duration of the follow-up.

• The Korean journal of internal medicine
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• v.33 no.6
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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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• v.16 no.15
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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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• v.51 no.5
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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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• v.10 no.1
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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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• v.49 no.3
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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.