Jae Joon Han
Blood Res 2023; 58(S1): S58-S65Abstract : Patients with chronic myeloid leukemia (CML) in the chronic phase receiving tyrosine kinase inhibitor (TKI) therapy are expected to have long-term survival outcomes comparable to those of the general population. Many clinical trials have confirmed that some patients sustain molecular responses without continuing TKI therapy. Treatment-free remission (TFR) is a new goal in treating chronic CML. The safety and outcome of TFR were studied in clinical trials after discontinuing imatinib or the second-generation TKIs dasatinib or nilotinib. TFR was safe in approximately 50% of patients who achieved a deep molecular response to TKI therapy. Patients who relapsed after discontinuing TKI responded immediately to the reintroduction of TKI. The mechanism by which TFR increases the success rate still needs to be understood. The hypothesis that the modulation of immune function and targeting of leukemic stem cells could improve the TFR is under investigation. Despite the remaining questions, the TFR has become a routine consideration for clinicians in the practice of molecular remission in patients with CML.
Abstract : Light chain (AL) amyloidosis is a disease in which malignant plasma cell clones affect multiple organs including the heart and kidney. The mechanism for organ function deterioration in AL amyloidosis differs from multiple myeloma. Thus, not all agents used to treat multiple myeloma shows similar efficacy in AL amyloidosis. In AL amyloidosis, both hematologic and organ responses after treatment are important to improve the clinical outcome. Especially, improving heart function is one of the key aspects in the treatment of AL amyloidosis. With recent advances in the understanding of the pathophysiologic mechanism of AL amyloidosis, novel treatment methods are under active trial. In this article, I have reviewed the advances in pathophysiology, diagnosis, risk stratification, and treatment of AL amyloidosis.
Jin Kyung Suh, Sunghan Kang, Hyery Kim, Ho Joon Im, Kyung-Nam Koh
Blood Res 2021; 56(S1): S65-S69Abstract : Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder caused by the clonal expansion of myeloid precursors that differentiate into CD1a+/CD207+ cells in the lesion. Advances in genomic sequencing techniques have improved our understanding of the pathophysiology of LCH. Activation of the mitogen-activated protein kinase (MAPK) pathway is a key molecular mechanism involved in the development of LCH. Recurrent BRAF mutations and MAP2K1 mutations are the major molecular alterations involved in the activation of the MAPK pathway. Recent studies have supported the “misguided myeloid differentiation model” of LCH, where the extent of disease is defined by the differentiation stage of the cell in which the activating somatic MAPK mutation occurs, suggesting LCH. Several studies have advocated the efficacy of targeted therapy using BRAF inhibitors with a high response rate, especially in patients with high-risk or refractory LCH. However, the optimal treatment scheme for children remains unclear. This review outlines recent advances in LCH, focusing on understanding the molecular pathophysiology, emerging targeted therapy options, and their clinical implications.
Abstract : Patients with inherited bone marrow failure syndrome (IBMFS) can develop peripheral blood cytopenia, which can ultimately progress to myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Although some cases of IBMFS are diagnosed based on their typical presentation, variable disease penetrance and expressivity may result in diagnostic dilemmas. With recent advances in genomic evaluation including next-generation sequencing, many suspected cases of IBMFS with atypical presentations can be identified. Identification of the genetic causes of IBMFS has led to important advances in understanding DNA repair, telomere biology, ribosome biogenesis, and hematopoietic stem cell regulation. An overview of this syndromes is summarized in this paper.
Sang Hyuk Park, Yoo Jin Lee, Youjin Kim, Hyun-Ki Kim, Ji-Hun Lim, Jae-Cheol Jo
Blood Res 2023; 58(S1): S52-S57Abstract : T-cell large granular lymphocyte (T-LGL) leukemia is characterized by clonal expansion of cytotoxic T cells resulting in cytopenia. The proliferation of clonal LGLs is caused by prolonged antigenic stimulation, which leads to apoptotic dysregulation owing mainly to the constitutive activation of survival pathways, notably the JAK/STAT pathway. Understanding how leukemic T-LGL persists can aid in the development of future immunosuppressive therapies. In this review, we summarize the diagnosis and current standard of therapy for T-LGL leukemia, as well as recent advances in clinical trials.
Sung-Hoon Jung, Youngil Koh, Min Kyoung Kim, Jin Seok Kim, Joon Ho Moon, Chang-Ki Min, Dok Hyun Yoon, Sung-Soo Yoon, Je-Jung Lee, Chae Moon Hong, Ka-Won Kang, Jihyun Kwon, Kyoung Ha Kim, Dae Sik Kim, Sung Yong Kim, Sung-Hyun Kim, Yu Ri Kim, Young Rok Do, Yeung-Chul Mun, Sung-Soo Park, Young Hoon Park, Ho Jin Shin, Hyeon-Seok Eom, Sang Eun Yoon, Sang Mee Hwang, Won Sik Lee, Myung-won Lee, Jun Ho Yi, Ji Yun Lee, Ji Hyun Lee, Ho Sup Lee, Sung-Nam Lim, Jihyang Lim, Ho-Young Yhim, Yoon Hwan Chang, Jae-Cheol Jo, Jinhyun Cho, Hyungwoo Cho, Yoon Seok Choi, Hee jeong Cho, Ari Ahn, Jong Han Choi, Hyun Jung Kim and Kihyun Kim
Blood Res 2025; 60():Abstract : Multiple myeloma (MM), a hematological malignancy, is characterized by malignant plasma cell proliferation in the bone marrow. Recent treatment advances have significantly improved patient outcomes associated with MM. In this study, we aimed to develop comprehensive, evidence-based guidelines for the diagnosis, prognosis, and treatment of MM. We identified 12 key clinical questions essential for MM management, guiding the extensive literature review and meta-analysis of the study. Our guidelines provide evidence-based recommendations by integrating patient preferences with survey data. These recommendations include current and emerging diagnostic tools, therapeutic agents, and treatment strategies. By prioritizing a patient-centered approach and rigorous data analysis, these guidelines were developed to enhance MM management, both in Korea and globally.
Erin-Siobhain R. Currin, and Ajay K. Gopal
Korean J Hematol 2012; 47(1): 8-16Abstract : Hodgkin lymphoma (HL) represents one of the great success stories in hematology going from a uniformly fatal disease, to one that is curable in the vast majority of cases. Despite this success, some patients experience relapse. To address this unmet need a variety of agents, classes of drugs, and strategies have demonstrated activity in HL recurring after autologous hematopoietic stem cell transplantation. These include chemotherapeutics (gemcitabine-based combinations, bendamustine), histone deacetylase (HDAC) inhibitors (panobinostat), immunomodulatory agents (lenalidomide), mTOR inhiobitors (everolimus), monoclonal antibodies (rituximab), and antibody-drug conjugates (brentuximab vedotin) as well the potential of long-term disease control via allogeneic transplantation. Such advances reflect our increased understanding of the biology of HL and hold promise for continued improved outcomes for those suffering with this condition.
Walter Ageno
Korean J Hematol 2010; 45(1): 8-13Abstract : Venous thromboembolism (VTE) is a spectrum of diseases that includes deep vein thrombosis (DVT) and pulmonary embolism (PE). Anticoagulant treatment is the mainstay of therapy for VTE. Unfractionated heparin (UFH) or low molecular weight heparin (LMWH) followed by vitamin K antagonists have been the treatment of choice for most patients with VTE, with the aim to prevent thrombus extension or embolization and recurrent VTE. Fondaparinux, a selective, indirect, parenteral factor Xa inhibitor, is now also approved for the initial treatment of VTE and represents an important alternative to UFH or LMWH. Secondary prevention of VTE with vitamin K antagonists is usually prescribed for a minimum of three months, with the duration of treatment based on the presence or absence of major identifiable risk factors for the index event. Patients with permanent risk factors or patients with recurrent DVT or PE require life long secondary prevention. Over the last years, new oral anticoagulant agents have been developed and are now undergoing extensive clinical evaluation in several settings, including the treatment of VTE. New oral anticoagulants include selective, direct thrombin inhibitors, such as dabigatran etexilate, and selective, direct factor Xa inhibitos, such as rivaroxaban, apixaban or edoxaban. All these drugs are admistered at fixed daily doses and do not require laboratory monitoring. The positive results of the first completed clinical trials suggest that a new era in the management of VTE is about to begin.
Ho Joon Im, Kyung-Nam Koh, and Jong Jin Seo
Blood Res 2016; 51(1): 8-16Abstract : Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative treatment for children and adolescents with various malignant and non-malignant diseases. While human leukocyte antigen (HLA)-identical sibling donor is the preferred choice, matched unrelated volunteer donor is another realistic option for successful HSCT. Unfortunately, it is not always possible to find a HLA-matched donor for patients requiring HSCT, leading to a considerable number of deaths of patients without undergoing transplantation. Alternatively, allogeneic HSCT from haploidentical family members could provide donors for virtually all patients who need HSCT. Although the early attempts at allogeneic HSCT from haploidentical family donor (HFD) were disappointing, recent advances in the effective
Ji Hyun Lee, Sung-Hyun Kim
Blood Res 2020; 55(S1): S43-S53Abstract : The therapeutic strategy for relapsed and refractory multiple myeloma (RRMM) integrates a holistic approach regarding patient, disease, and drug-related factors. Patient-related factors include age, frailty status, and underlying comorbidities, especially cardiovascular and renal diseases and peripheral neuropathies that affect tolerability to multiple drug combinations or transplantations. Disease-related factors encompass these multiple patient-related factors, particularly the aggressiveness of the disease and cytogenetics. Regarding drug-related factors, the approval of novel proteasome inhibitors (such as carfilzomib and ixazomib), immunomodulatory agents (such as pomalidomide), monoclonal antibodies (such as daratumumab and elotuzumab), and new classes of drugs increasingly makes the choice treatment more complex and necessitates a comprehensive summary and an update of the efficacy and toxicities of each antimyeloma drug and its combinations. Further, careful monitoring of the side effects and supportive care throughout the course of treatment are important to achieve better outcomes for patients with RRMM.
Christopher Chin Keong Liam, Jim Yu-Hsiang Tiao, Yee Yee Yap, Yi Lin Lee, Jameela Sathar, Simon McRae, Amanda Davis, Jennifer Curnow, Robert Bird, Philip Choi, Pantep Angchaisuksiri, Sim Leng Tien, Joyce Ching Mei Lam, Doyeun Oh, Jin Seok Kim, Sung-Soo Yoon, Raymond Siu-Ming Wong, Carolyn Lauren, Eileen Grace Merriman, Anoop Enjeti, Mark Smith, Ross Ian Baker
Blood Res 2023;58: 36-41Dong Wook Jekarl, Jae Kwon Kim, Jay Ho Han, Howon Lee, Jaeeun Yoo, Jihyang Lim, Yonggoo Kim
Blood Res 2023;58: S1-S7Yoo Jin Lee, Youjin Kim, Sang Hyuk Park, Jae-Cheol Jo
Blood Res 2023;58: S90-S95Eun-Ji Choi
Blood Res 2023;58: S29-S36+82-2-516-6582