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Abstract : Chronic lymphocytic leukemia is a genetically heterogeneous disease, and a complex set of genetic alterations is associated with its pathogenesis. CLL is the most common leukemia in the western countries, whereas it is rare in Asia, including Korea. The prognostic models integrate the traditional staging systems developed by Rai et al. and Binet et al. with biochemical and genetic markers. With the advent of molecular biology, a variety of targeted agents, including anti-CD20 antibodies, inhibitors of BCR signaling pathway, and BCL-2 inhibitors, have been introduced, which has changed the landscape of CLL treatment greatly. This review will focus on the risk stratification and the management of CLL in the era of novel small molecules.

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Abstract : A bibliometric study is performed to analyze publication patterns in a specific research area and to establish a landscape model that can be used to quantitatively weigh publications. This study aimed to investigate AML research networks and to conduct a trend-related keyword analysis. We analyzed 48,202 studies about AML published from 1999 to 2019 in the Web of Science Core Collection. The network analysis was conducted using the R&R studio software. The journal Blood had the highest number of published articles with an h-index of 410. The USA had the highest number of total publications (18,719, 38.3%) and research funded by the government, institutions, and pharmaceutical companies (5,436, 10.8%). The institute with the largest number of publications was the MD Anderson Cancer Center. Kantarjian H, Garcia-Manero G, and Ravandi F were the leading authors of publications about AML. Keyword analysis revealed that FLT 3, micro-RNA, and NK cell topics were the hotspots in the cell and gene area in all publications. The overall AML research landscape is popular in the field of translational research as it can identify molecular, cell, and gene studies conducted by different funding agencies, countries, institutions, and author networks. With active funding and support from the Chinese government, the productivity of scientific research is increasing not only in the AML field but also in the medical/health-related science field.

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Abstract : Acute myeloblastic leukemia (AML) is the most frequent acute leukemia in adulthood with very poor overall survival rates. In the past few decades, significant progresses had led to the findings of new therapeutic approaches and the better understanding of the molecular complexity of this hematologic malignancy. Leukemic stem cells (LSCs) play a key role in the initiation, progression, regression, and drug resistance of different types of leukemia. The cellular and molecular characteristics of LSCs and their mechanism in the development of leukemia had not yet been specified. Therefore, determining their cellular and molecular characteristics and creating new approaches for targeted therapy of LSCs is crucial for the future of leukemia research. For this reason, the recognition of surface maker targets on the cell surface of LSCs has attracted much attention. CD33 has been detected on blasts in most AML patients, making them an interesting target for AML therapy. Genetic engineering of T cells with chimeric antigen receptor (CAR-T cell therapy) is a novel therapeutic strategy. It extends the range of antigens available for use in adoptive T-cell immunotherapy. This review will focus on CAR-T cell approaches as well as monoclonal antibody (mAB)-based therapy, the two antibody-based therapies utilized in AML treatment.

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Abstract : Drug resistance in cancer, especially in leukemia, creates a dilemma in treatment planning. Consequently, studies related to the mechanisms underlying drug resistance, the molecular pathways involved in this phenomenon, and alternate therapies have attracted the attention of researchers. Among a variety of therapeutic modalities, mesenchymal stem cells (MSCs) are of special interest due to their potential clinical use. Therapies involving MSCs are showing increasing promise in cancer treatment and anticancer drug screening applications; however, results have been inconclusive, possibly due to the heterogeneity of MSC populations. Most recently, the effect of MSCs on different types of cancer, such as hematologic malignancies, their mechanisms, sources of MSCs, and its advantages and disadvantages have been discussed. There are many proposed mechanisms describing the effects of MSCs in hematologic malignancies; however, the most commonly-accepted mechanism is that MSCs induce tumor cell cycle arrest. This review explains the anti-tumorigenic effects of MSCs through the suppression of tumor cell proliferation in hematological malignancies, especially in acute myeloid leukemia.

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Abstract : An increase in biochemical concentrations of non-transferrin bound iron (NTBI) within the patients with an increase in serum iron concentration was evaluated with the following objectives: (a) Iron overloading diseases/conditions with free radicle form of ‘iron containing’ reactive oxygen species (ROS) and its imbalance mediated mortality, and (b) Intervention with iron containing drugs in context to increased redox iron concentration and treatment induced mortality. Literature search was done within Pubmed and cochrane review articles. The Redox iron levels are increased during dys-erythropoiesis and among transfusion recipient population and are responsive to iron-chelation therapy. Near expiry ‘stored blood units’ show a significant rise in the ROS level. Iron mediated ROS damage may be estimated by the serum antioxidant level, and show reduction in toxicity with high antioxidant, low pro-oxidant levels. Iron drug therapy causes a significant increase in NTBI and labile iron levels. Hospitalized patients on iron therapy however show a lower mortality rate. Serum ferritin is a mortality indicator among the high-dose iron therapy and transfusion dependent population. The cumulative difference of pre-chelation to post chelation ROS iron level was 0.97 (0.62; 1.32; N=261) among the transfusion dependent subjects and 2.89 (1.81–3.98; N=130) in the post iron therapy ‘iron ROS’ group. In conclusion, iron mediated mortality may not be mediated by redox iron among multi-transfused and iron overloaded patients.

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Abstract : Iron deficiency anemia and anemia of chronic disorders are the most common types of anemia. Disorders of iron metabolism lead to different clinical scenarios such as iron deficiency anemia, iron overload, iron overload with cataract and neurocognitive disorders. Regulation of iron in the body is a complex process and different regulatory proteins are involved in iron absorption and release from macrophages into hematopoietic tissues. Mutation in these regulatory genes is the most important cause of iron refractory iron deficiency anemia (IRIDA). This review provides a glance into the iron regulation process, diseases related to iron metabolism, and appropriate treatments at the molecular level.

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Abstract : Genetic hemoglobin disorders are caused by mutations and/or deletions in the α-globin or β-globin genes. Thalassemia is caused by quantitative defects and hemoglobinopathies by structural defect of hemoglobin. The incidence of thalassemia and hemoglobinopathy is increased in Korea with rapid influx of people from endemic areas. Thus, the awareness of the disease is needed. α-thalassemias are caused by deletions in α-globin gene, while β-thalassemias are associated with decreased synthesis of β-globin due to β-globin gene mutations. Hemoglobinopathies involve structural defects in hemoglobin due to altered amino acid sequence in the α- or β-globin chains. When the patient is suspected with thalassemia/hemoglobinopathy from abnormal complete blood count findings and/or family history, the next step is detecting hemoglobin abnormality using electrophoresis methods including high performance liquid chromatography and mass spectrometry. The development of novel molecular genetic technologies, such as massively parallel sequencing, facilitates a more precise molecular diagnosis of thalassemia/hemoglobinopathy. Moreover, prenatal diagnosis using genetic testing enables the prevention of thalassemia birth and pregnancy complications. We aimed to review the spectrum and classification of thalassemia/hemoglobinopathy diseases and the diagnostic strategies including screening tests, molecular genetic tests, and prenatal diagnosis.

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Abstract : Coronary artery ectasia (CAE) is defined as the dilation of a segment of a coronary vessel to at least 1.5 times the diameter of its normal adjacent segment. Mean platelet volume (MPV) plays a role in acute coronary syndromes, with high MPV correlating to poor prognosis for acute thrombotic events and CAE. Several studies investigated the relationship between MPV and CAE, resulting in conflicting results. These results led us to systematically review all studies investigating the relationship between MPV and ectatic heart diseases by performing a meta-analysis study in order to report a unified result. This meta-analysis study investigated all case-control articles examining the relationship between MPV and CAE. All studies in the following databases published until January 31, 2018, were investigated: Science Direct, Scopus, PubMed, Google Scholar, and Web of Science. Following a quality control evaluation, 14 articles, all of which were published following studies performed in Turkey from 2007 to 2016, met the criteria for study inclusion. After pooling the results from all of the articles, a total standardized mean difference (SMD) value of 0.584 (95% CI, 0.219, 0.95) was obtained using the D+L pooled SMD, indicating a significant difference (P=0.002) between the two groups, with higher MPV values in ectatic patients when comparing to healthy individuals. Therefore, increased MPV levels were significantly related to CAE, suggesting that platelets, with their inflammatory and thrombotic activities, play a role in this disease. Therefore, anti-platelet and anti-inflammatory therapies may be effective in treating CAE.

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Abstract : Brentuximab vedotin (BV), a potent antibody-drug conjugate, targets the CD30 antigen. Owing to the remarkable efficacy shown in CD30-positive lymphomas, such as Hodgkin's lymphoma and systemic anaplastic large-cell lymphoma, BV was granted accelerated approval in 2011 by the US Food and Drug Administration. Thereafter, many large-scale trials in various situations have been performed, which led to extensions of the original indication. The aim of this review was to describe the latest updates on clinical trials of BV and the in-practice guidance for the use of BV.

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Abstract : Management options for patients with immune thrombocytopenia (ITP) have evolved substantially over the past decades. The American Society of Hematology published a treatment guideline for clinicians referring to the management of ITP in 2011. This evidence-based practice guideline for ITP enables the appropriate treatment of a larger proportion of patients and the maintenance of normal platelet counts. Korean authority operates a unified mandatory national health insurance system. Even though we have a uniform standard guideline enforced by insurance reimbursement, there are several unsolved issues in real practice in ITP treatment. To optimize the management of Korean ITP patients, the Korean Society of Hematology Aplastic Anemia Working Party (KSHAAWP) reviewed the consensus and the Korean data on the clinical practices of ITP therapy. Here, we report a Korean expert recommendation guide for the management of ITP.