The Korean Journal of Hematology最新文献_第10页

New clinical score for disease activity at diagnosis in Langerhans cell histiocytosis. 朗格汉斯组织细胞增多症诊断时疾病活动性的新临床评分。

The Korean Journal of Hematology Pub Date : 2011-09-01 Epub Date: 2011-09-30 DOI: 10.5045/kjh.2011.46.3.186

Won-Ik Choi, You Cheol Jeong, Sun Young Kim, So Dam Kim, John Paul Pribis, Hee-Jin Kim, Kyung-Nam Koh, Ho-Joon Im, Young-Ho Lee, Jong-Jin Seo

{"title":"New clinical score for disease activity at diagnosis in Langerhans cell histiocytosis.","authors":"Won-Ik Choi, You Cheol Jeong, Sun Young Kim, So Dam Kim, John Paul Pribis, Hee-Jin Kim, Kyung-Nam Koh, Ho-Joon Im, Young-Ho Lee, Jong-Jin Seo","doi":"10.5045/kjh.2011.46.3.186","DOIUrl":"https://doi.org/10.5045/kjh.2011.46.3.186","url":null,"abstract":"Background: The clinical presentation and course of Langerhans cell histiocytosis (LCH) are variable, ranging from an isolated, spontaneously remitting bone lesion to multisystem disease with risk organ involvement. Treatment of LCH ranges from a wait-and-see attitude to intensive multidrug therapy and, in some cases, bone marrow transplantation. It is necessary to develop an objective score for assessing disease activity in patients with LCH. We propose a new clinical scoring system to evaluate disease activity at diagnosis that can predict the clinical outcomes of LCH and correlate it with clinical courses.Methods: Clinical data, obtained from children diagnosed with LCH at Asan Medical Center and Hanyang University Hospital between March 1998 and February 2009, were studied retrospectively. The scoring system was developed according to the basic biological data, radiological findings, and physical findings and applied to a database containing information on 133 patients.Results: The median age of the 133 patients (74 male, 59 female) was 52 months (range, 0.6-178 months), and LCH was diagnosed based on CD1a positivity. At diagnosis, the score distributions were highly asymmetrical: the score was between 1 and 2 in 75.9% of cases, 3-6 in 15.8%, and greater than 6 in 8.3%. Initial scores above 6 were highly predictive of reactivation and late complications.Conclusion: This new LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow-up.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"46 3","pages":"186-91"},"PeriodicalIF":0.0,"publicationDate":"2011-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2011.46.3.186","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40138109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 3

Genotypic influence of α-deletions on the phenotype of Indian sickle cell anemia patients. α-缺失对印度镰状细胞性贫血患者表型的基因型影响

The Korean Journal of Hematology Pub Date : 2011-09-01 Epub Date: 2011-09-30 DOI: 10.5045/kjh.2011.46.3.192

Sanjay Pandey, Sweta Pandey, Rahasya Mani Mishra, Monica Sharma, Renu Saxena

{"title":"Genotypic influence of α-deletions on the phenotype of Indian sickle cell anemia patients.","authors":"Sanjay Pandey, Sweta Pandey, Rahasya Mani Mishra, Monica Sharma, Renu Saxena","doi":"10.5045/kjh.2011.46.3.192","DOIUrl":"10.5045/kjh.2011.46.3.192","url":null,"abstract":"Background: Some reports have shown that co-inheritance of α-thalassemia and sickle cell disease improves hematological parameters and results in a relatively mild clinical picture for patients; however, the exact molecular basis and clinical significance of the interaction between α-thalassemia and sickle cell disease in India has not yet been described. There is little agreement on the clinical effects of α-thalassemia on the phenotype of sickle cell disease.Methods: Complete blood count and red cell indices were measured by an automated cell analyzer. Quantitative assessment of hemoglobin variants HbF, HbA, HbA(2), and HbS was performed by high performance liquid chromatography (HPLC). DNA extraction was performed using the phenol-chloroform method, and molecular study for common α-deletions was done by gap-PCR.Results: Out of 60 sickle cell anemia patients, the α-thalassemia genotype was found in 18 patients. Three patients had the triplicated α-genotype (Anti α-3.7 kb), and the remaining patients did not have α-deletions. This study indicates that patients with co-existing α-thalassemia and sickle cell disease had a mild phenotype, significantly improved hematological parameters, and fewer blood transfusions than the patients with sickle cell anemia without co-existing α-deletions.Conclusion: Co-existence of α-thalassemia and sickle cell anemia has significant effects on the phenotype of Indian sickle cell patients.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"46 3","pages":"192-5"},"PeriodicalIF":0.0,"publicationDate":"2011-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2011.46.3.192","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40137508","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 25

Treatment of chronic graft-versus-host disease: Past, present and future. 慢性移植物抗宿主病的治疗:过去、现在和未来。

The Korean Journal of Hematology Pub Date : 2011-09-01 Epub Date: 2011-09-30 DOI: 10.5045/kjh.2011.46.3.153

Paul J Martin, Yoshihiro Inamoto, Paul A Carpenter, Stephanie J Lee, Mary E D Flowers

{"title":"Treatment of chronic graft-versus-host disease: Past, present and future.","authors":"Paul J Martin, Yoshihiro Inamoto, Paul A Carpenter, Stephanie J Lee, Mary E D Flowers","doi":"10.5045/kjh.2011.46.3.153","DOIUrl":"https://doi.org/10.5045/kjh.2011.46.3.153","url":null,"abstract":"Chronic GVHD was recognized as a complication of allogeneic hematopoietic cell transplantation more than 30 years ago, but progress has been slowed by the limited insight into the pathogenesis of the disease and the mechanisms that lead to development of immunological tolerance. Only 6 randomized phase III treatment studies have been reported. Results of retrospective studies and prospective phase II clinical trials suggested overall benefit from treatment with mycophenolate mofetil or thalidomide, but these results were not substantiated by phase III studies of initial systemic treatment for chronic GVHD. A comprehensive review of published reports showed numerous deficiencies in studies of secondary treatment for chronic GVHD. Fewer than 10% of reports documented an effort to minimize patient selection bias, used a consistent treatment regimen, or tested a formal statistical hypothesis that was based on a contemporaneous or historical benchmark. In order to enable valid comparison of the results from different studies, eligibility criteria, definitions of individual organ and overall response, and time of assessment should be standardized. Improved treatments are more likely to emerge if reviewers and journal editors hold authors to higher standards in evaluating manuscripts for publication.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"46 3","pages":"153-63"},"PeriodicalIF":0.0,"publicationDate":"2011-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2011.46.3.153","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40138104","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 10

Recent advances in the path toward the cure for chronic myeloid leukemia. 慢性髓性白血病治疗途径的最新进展。

The Korean Journal of Hematology Pub Date : 2011-09-01 Epub Date: 2011-09-30 DOI: 10.5045/kjh.2011.46.3.169

Dong-Wook Kim

{"title":"Recent advances in the path toward the cure for chronic myeloid leukemia.","authors":"Dong-Wook Kim","doi":"10.5045/kjh.2011.46.3.169","DOIUrl":"https://doi.org/10.5045/kjh.2011.46.3.169","url":null,"abstract":"Through the phase 3 International Randomized Study of Interferon vs. STI571 (IRIS) trial, imatinib emerged as the standard treatment for chronic myeloid leukemia (CML) and has successfully prolonged the duration of both the chronic phase (CP) and the disease-free state. The majority of newly diagnosed patients treated for CP-CML achieve a complete cytogenetic response (CCyR), and over time, most of these eventually achieve major molecular responses (MMRs) and even complete molecular responses (CMRs). In ongoing phase 3 randomized trials of second-generation tyrosine kinase inhibitors (TKIs), nilotinib and dasatinib have been found to have superior efficacies in helping achieve cytogenetic and molecular responses, including MMRs and CMRs. However, only the MMR rate was significantly higher in bosutinib compared with the imatinib control, but not in CCyR rate. Current reports of imatinib discontinuation suggested that achieving CMR is an important prerequisite for CML to be cured. Recent data from the STIM (Stop Imatinib) trial showed that imatinib can be successfully discontinued in patients who achieve a certain level of CMR. Standardized real-time quantitative reverse transcriptase-polymerase chain reaction (RQ-PCR) assays have been available in routine clinical practice, and efforts are being focused on achieving higher sensitivity and optimizing the time of imatinib discontinuation. Although very few patients are cured by administration of only Bcr-Abl TKIs, including imatinib and second-generation TKIs, current advances may eventually make this possible. This report summarizes the detailed clinical data obtained in the DASISION, ENESTnd, and BELA studies and discusses high-sensitivity detection methods and future therapeutic strategies.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"46 3","pages":"169-74"},"PeriodicalIF":0.0,"publicationDate":"2011-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2011.46.3.169","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40138106","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 6

The pathophysiology of chronic graft-versus-host disease: the unveiling of an enigma. 慢性移植物抗宿主病的病理生理学:一个谜的揭开。

The Korean Journal of Hematology Pub Date : 2011-06-01 Epub Date: 2011-06-21 DOI: 10.5045/kjh.2011.46.2.80

Chang-Ki Min

{"title":"The pathophysiology of chronic graft-versus-host disease: the unveiling of an enigma.","authors":"Chang-Ki Min","doi":"10.5045/kjh.2011.46.2.80","DOIUrl":"https://doi.org/10.5045/kjh.2011.46.2.80","url":null,"abstract":"Chronic graft-versus-host disease (CGVHD) is one of the most significant complications of long-term survivors after allogeneic hematopoietic stem cell transplantation (allo-HSCT). CGVHD may have protean manifestations and can pose unique diagnostic and therapeutic challenges. New recommendations that emphasize the importance of qualitative differences, as opposed to time of onset after HSCT, are now being used to standardize the diagnosis and clinical assessment of CGVHD, but they require validation. During the past 3 decades, experimental studies and clinical observations have elucidated the mechanisms of acute GVHD, but its biology is much less well-understood. Experimental studies have generated at least 4 theories to explain the pathophysiology of CGVHD: (1) thymic damage and the defective negative selection of T cells, (2) regulatory T cell deficiencies, (3) auto-antibody production by aberrant B cells, and (4) the formation of profibrotic lesions. Mouse models have provided important insights into the pathophysiology of CGVHD, and these have helped improve clinical outcomes following allo-HSCT, but no animal model fully replicates all of the features of CGVHD in humans. In this article, recent clinical changes, the pathogenesis of CGHVD, the cellular and cytokine networks implicated in its pathogenesis, and the animal models used to devise strategies to prevent and treat CGVHD are reviewed.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"46 2","pages":"80-7"},"PeriodicalIF":0.0,"publicationDate":"2011-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2011.46.2.80","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"29998429","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 47

Hemoglobin F level in different hemoglobin variants. 不同血红蛋白变体的血红蛋白F水平。

The Korean Journal of Hematology Pub Date : 2011-06-01 Epub Date: 2011-06-21 DOI: 10.5045/kjh.2011.46.2.118

Akanni E Olufemi, Oseni B Sola, Bamisaye E Oluwaseyi, Raji A Ajani, Mewoyeka O Olusoji, Hassan R Olubunmi

{"title":"Hemoglobin F level in different hemoglobin variants.","authors":"Akanni E Olufemi, Oseni B Sola, Bamisaye E Oluwaseyi, Raji A Ajani, Mewoyeka O Olusoji, Hassan R Olubunmi","doi":"10.5045/kjh.2011.46.2.118","DOIUrl":"https://doi.org/10.5045/kjh.2011.46.2.118","url":null,"abstract":"Background: Fetal hemoglobin (HbF) levels in different hemoglobin variants in Osogbo, Nigeria, were estimated using two principal methods of estimation using existing information for HbF concentration and distribution of various hemoglobin variants in the area, as well as diagnosed cases of thalassemia. Two hundred and sixty samples collected from HbSS, HbSC, HbAA, HbAS, and HbAC subjects were analyzed. HbF level and hemoglobin type were determined in this study.Methods: The hemoglobin type was determined using cellulose acetate electrophoresis at an alka-line pH, and HbF was determined by the acid elution and alkaline denaturation methods.Results: The mean±SD of HbF in the respective hemoglobin variants was as follows: HbSS, 2.09±1.94%; HbSC, 0.85±0.54%; HbAA, 0.69±0.46%; HbAS, 0.52±0.31%; and HbAC, 0.57±0.26%. The mean HbF level across the hemoglobin variants was statistically significant (P<0.05). Investigating the sex distribution of the HbF level in the studied population revealed a slightly higher mean HbF level in females than in their male counterparts.Conclusion: Within the study population, the HbF level was found to be highest in HbSS and lowest in HbAS. The two methods of estimating HbF are equally reliable, since there was no significant difference between the results obtained from the two methods.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"46 2","pages":"118-22"},"PeriodicalIF":0.0,"publicationDate":"2011-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2011.46.2.118","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"29999497","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 8

Successful treatment with erythromycin for idiopathic thrombocytopenic purpura. 红霉素成功治疗特发性血小板减少性紫癜。

The Korean Journal of Hematology Pub Date : 2011-06-01 Epub Date: 2011-06-21 DOI: 10.5045/kjh.2011.46.2.139

Masashi Ohe, Satoshi Hashino

引用次数: 15

Cherishing the memory of Professor Sang-In Kim 怀念金相仁教授

The Korean Journal of Hematology Pub Date : 2011-06-01 DOI: 10.5045/KJH.2011.46.2.59

S. Kwon

{"title":"Cherishing the memory of Professor Sang-In Kim","authors":"S. Kwon","doi":"10.5045/KJH.2011.46.2.59","DOIUrl":"https://doi.org/10.5045/KJH.2011.46.2.59","url":null,"abstract":"Prof. Sang-In Kim would have been greatly proud and happy to see us advancing the Laboratory Medicine if he could have stayed with us longer. We feel the deepest sorrow after he had left us so soon. Having strongly endured the long fight against the disease, he even attended the transfusion conference several months ago, there he smiled and grabbed our hands with his warm heart. On the day he was leaving, the spring snow was falling and the cold rain was sprinkling all over the land, mixing all with our sad tears. He was the pioneer and godfather of Laboratory Medicine. He had laid the foundation stones on hematology and transfusion medicine in Korea. Born in a small village of southern province of Korea in 1928, he studied in Busan at the time of Japanese annexation. Having graduated from Seoul National University College of Medicine in 1955, he became a doctor with full of dreams. After studying in the pathology laboratories of University of Minnesota, he came back to homeland and worked hard even in adverse circumstances, and accomplished countless academic achievements which set excellent models for us all. Always being generous, benevolent, and venerable, he led us with his love and planted passion and challenging spirit within our hearts. Now we are being raised on the foundation stones that he had laid. In many universities and hospitals, we are following his path and working hard to build better Laboratory Medicine. We now wish him a peaceful journey. Just like returning from a picnic from this beautiful world, fly well to the heaven and please say that it was beautiful. We dedicate our love and respect to our great teacher.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"16 1","pages":"59 - 59"},"PeriodicalIF":0.0,"publicationDate":"2011-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90237824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Gametocytes of Plasmodium falciparum in the megakaryocytes. 恶性疟原虫巨核细胞中的配子体。

The Korean Journal of Hematology Pub Date : 2011-06-01 Epub Date: 2011-06-21 DOI: 10.5045/kjh.2011.46.2.68

Harish Chandra, Smita Chandra

引用次数: 1

Treatment outcomes in children with Burkitt lymphoma and L3 acute lymphoblastic leukemia treated using the lymphoma malignancy B protocol at a single institution. 伯基特淋巴瘤和L3急性淋巴细胞白血病患儿在单一机构使用恶性淋巴瘤B方案的治疗结果

The Korean Journal of Hematology Pub Date : 2011-06-01 Epub Date: 2011-06-21 DOI: 10.5045/kjh.2011.46.2.96

Eun Sil Park, Hyery Kim, Ji Won Lee, Jae-Young Lim, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin, Hyo Seop Ahn

{"title":"Treatment outcomes in children with Burkitt lymphoma and L3 acute lymphoblastic leukemia treated using the lymphoma malignancy B protocol at a single institution.","authors":"Eun Sil Park, Hyery Kim, Ji Won Lee, Jae-Young Lim, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin, Hyo Seop Ahn","doi":"10.5045/kjh.2011.46.2.96","DOIUrl":"https://doi.org/10.5045/kjh.2011.46.2.96","url":null,"abstract":"Background: We compared the outcomes of patients with Burkitt lymphoma and French-American-British (FAB) L3 acute lymphoblastic leukemia treated using Lymphoma Malignancy B (LMB) or other treatment protocols.Methods: Thirty-eight patients diagnosed between July 1996 and December 2007 were treated using LMB 96, and 22 patients diagnosed between January 1991 and May 1998 (defined as the early period) were treated using the D-COMP or CCG-106B protocols. We retrospectively reviewed their medical records and analyzed cumulative survival according to the treatment period by using Kaplan-Meier analysis.Results: There were no intergroup differences in the distribution of age, disease stage, or risk group. The median follow-up period of the 33 live patients in the LMB group was 72 months (range, 36-170 months). Overall survival (OS) and event-free survival (EFS) of patients treated using LMB 96 were 86.8%±5.5% and 81.6%±6.3%, respectively, whereas OS and EFS of patients treated in the early period were 72.7%±9.6% and 68.2%±9.9%, respectively. In the LMB 96 group, OS of cases showing non-complete response (N=8) was 62.5%±17.1%, and OS of relapsed or primary refractory cases (N=6) was 33.3%±19.3%. Central nervous system (CNS) disease, high lactate dehydrogenase levels at diagnosis, and treatment response were significant prognostic factors.Conclusion: Survival outcome has drastically improved over the last 2 decades with short-term, dose-intensive chemotherapy. However, CNS involvement or poor response to chemotherapy was worse prognostic factors; therefore, future studies addressing this therapeutic challenge are warranted.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"46 2","pages":"96-102"},"PeriodicalIF":0.0,"publicationDate":"2011-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2011.46.2.96","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"29998431","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 7