Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10314061
F. McClanahan, J. Gribben
While the standard of care for chronic lymphocytic leukaemia (CLL) leads to high overall response rates and a long progression-free survival, it can be highly toxic for many patients, particularly in the elderly who often present concurrent diseases with associated morbidities. Treatment-related immune system burden and complications are challenging as most CLL patients already show immunodeficiency and are at high risk of infection. The latter are the main cause for increased morbidity and mortality and are correlated with disease severity and type of therapy. In the last few years, many new approaches and innovative agents such as second-generation anti-CD20 monoclonal antibodies, lenalidomide, B cell receptor signalling inhibitors, and novel cellular therapies have advanced the outlook for CLL management. Indeed, novel therapies could soon be addressing the need to promote immune reactivation and re-sensitise the immune system. By doing so, they could reach two main objectives, namely lowering the high proportion of patients at risk of infection, and acting as effective tools for the immune system to overcome its defects and fight malignant cells.
{"title":"Overcoming Immunodeficiency in Chronic Lymphocytic Leukaemia: Current Knowledge and Perspectives","authors":"F. McClanahan, J. Gribben","doi":"10.33590/emjhematol/10314061","DOIUrl":"https://doi.org/10.33590/emjhematol/10314061","url":null,"abstract":"While the standard of care for chronic lymphocytic leukaemia (CLL) leads to high overall response rates and a long progression-free survival, it can be highly toxic for many patients, particularly in the elderly who often present concurrent diseases with associated morbidities. Treatment-related immune system burden and complications are challenging as most CLL patients already show immunodeficiency and are at high risk of infection. The latter are the main cause for increased morbidity and mortality and are correlated with disease severity and type of therapy. In the last few years, many new approaches and innovative agents such as second-generation anti-CD20 monoclonal antibodies, lenalidomide, B cell receptor signalling inhibitors, and novel cellular therapies have advanced the outlook for CLL management. Indeed, novel therapies could soon be addressing the need to promote immune reactivation and re-sensitise the immune system. By doing so, they could reach two main objectives, namely lowering the high proportion of patients at risk of infection, and acting as effective tools for the immune system to overcome its defects and fight malignant cells.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127514634","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}
Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10312094
M. Dal Bo, E. Tissino, D. Benedetti, Chiara Caldana, R. Bomben, G. del Poeta, G. Gaidano, F. Rossi, A. Zucchetto, V. Gattei
Chronic lymphocytic leukaemia (CLL) is a clinically heterogeneous disease characterised by the accumulation/expansion of a clonal population of neoplastic cells with the morphological appearance of small mature B lymphocytes in blood, bone marrow, and lymphoid organs. Stimulation through the B cell receptor (BCR) plays a prominent role in the selection and expansion of the malignant clone in CLL. On the other hand, other external signals delivered by several cell types including T lymphocytes, macrophages, stromal cells, endothelial cells, and follicular dendritic cells, operating through either direct BCR-independent cell-cell contact or indirect production of paracrine soluble factors, synergistically cooperate in regulating proliferation and survival of CLL cells. In this context, CD49d is known to play a pivotal role in mediating both cell-cell and cell-matrix interactions in CLL-involved tissues, eventually delivering pro-survival signals and protecting CLL cells from drug-induced damages. In the present review, we focused on functional and physical interactions of CD49d with other microenvironmental receptors, including CD38 and BCR, and other specific CD49d-dependent interactions in lymph node and bone marrow microenvironments responsible for growth and survival-supporting signals, eventually influencing CLL prognosis and therapeutic options.
{"title":"The Role of CD49d in Chronic Lymphocytic Leukaemia: Microenvironmental Interactions and Clinical Relevance","authors":"M. Dal Bo, E. Tissino, D. Benedetti, Chiara Caldana, R. Bomben, G. del Poeta, G. Gaidano, F. Rossi, A. Zucchetto, V. Gattei","doi":"10.33590/emjhematol/10312094","DOIUrl":"https://doi.org/10.33590/emjhematol/10312094","url":null,"abstract":"Chronic lymphocytic leukaemia (CLL) is a clinically heterogeneous disease characterised by the accumulation/expansion of a clonal population of neoplastic cells with the morphological appearance of small mature B lymphocytes in blood, bone marrow, and lymphoid organs. Stimulation through the B cell receptor (BCR) plays a prominent role in the selection and expansion of the malignant clone in CLL. On the other hand, other external signals delivered by several cell types including T lymphocytes, macrophages, stromal cells, endothelial cells, and follicular dendritic cells, operating through either direct BCR-independent cell-cell contact or indirect production of paracrine soluble factors, synergistically cooperate in regulating proliferation and survival of CLL cells. In this context, CD49d is known to play a pivotal role in mediating both cell-cell and cell-matrix interactions in CLL-involved tissues, eventually delivering pro-survival signals and protecting CLL cells from drug-induced damages. In the present review, we focused on functional and physical interactions of CD49d with other microenvironmental receptors, including CD38 and BCR, and other specific CD49d-dependent interactions in lymph node and bone marrow microenvironments responsible for growth and survival-supporting signals, eventually influencing CLL prognosis and therapeutic options.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121700746","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}
Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10311182
P. Tosi
Autologous stem cell transplantation (ASCT) is considered the standard of care for multiple myeloma patients aged <65 years with no relevant comorbidities. The addition of proteasome inhibitors and/or immunomodulatory drugs has significantly increased the percentage of patients achieving a complete remission after induction therapy, and these results are maintained after high-dose melphalan (Alkeran®), leading to a prolonged disease control. Studies are being carried out in order to evaluate whether short- term consolidation or long-term maintenance therapy can result in disease eradication at the molecular level, thus also increasing patient survival. The efficacy of these new drugs has raised the issue of deferring the transplant after achieving a second response upon relapse. Another controversial point is the optimal treatment strategy for high-risk patients, that do not benefit from ASCT, and for whom the efficacy of new drugs is still matter of debate.
{"title":"Autologous Stem Cell Transplantation In Multiple Myeloma: Is It Still The Right Choice?","authors":"P. Tosi","doi":"10.33590/emjhematol/10311182","DOIUrl":"https://doi.org/10.33590/emjhematol/10311182","url":null,"abstract":"Autologous stem cell transplantation (ASCT) is considered the standard of care for multiple myeloma patients aged <65 years with no relevant comorbidities. The addition of proteasome inhibitors and/or immunomodulatory drugs has significantly increased the percentage of patients achieving a complete remission after induction therapy, and these results are maintained after high-dose melphalan (Alkeran®), leading to a prolonged disease control. Studies are being carried out in order to evaluate whether short- term consolidation or long-term maintenance therapy can result in disease eradication at the molecular level, thus also increasing patient survival. The efficacy of these new drugs has raised the issue of deferring the transplant after achieving a second response upon relapse. Another controversial point is the optimal treatment strategy for high-risk patients, that do not benefit from ASCT, and for whom the efficacy of new drugs is still matter of debate.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115264584","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}
Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10311247
M. Delforge, S. Knop, M. Mohty
In the last decades, advances in the therapeutic management of multiple myeloma (MM) with new drug armamentarium and strategies have significantly improved the outcome and survival of newly diagnosed and relapsed patients. However, the continuing challenges physicians are facing within specific clinical settings and patient subpopulations, whose prognosis with current strategies is extremely poor, call for a paradigm change. New immunomodulators, proteasome inhibitors, histone deacetylase inhibitors, and monoclonal antibodies are being explored to improve first-line outcomes so that a smaller proportion of patients relapse early or fail to respond to induction treatment. Moreover, recent advances and clinical evidence with novel therapies seem to provide patients with relapsed or refractory MM additional survival benefits. Improving clinical outcomes and refining standard of care should help clinicians reduce the burden of multiple and toxic therapy; quality of life (QoL) should be at the core of MM management. Patient selection and stratification needs to be reinforced with the help of comprehensive knowledge on conventional risk factors, and supplemented by molecular pathways in the near future in order to provide tailored options and strategies to patients, including the use of monoclonal antibodies. Numerous drugs are on the horizon and the next few years should witness marked improvements in survival, QoL, and safety of MM management.
{"title":"Current Developments and Perspectives in Multiple Myeloma","authors":"M. Delforge, S. Knop, M. Mohty","doi":"10.33590/emjhematol/10311247","DOIUrl":"https://doi.org/10.33590/emjhematol/10311247","url":null,"abstract":"In the last decades, advances in the therapeutic management of multiple myeloma (MM) with new drug armamentarium and strategies have significantly improved the outcome and survival of newly diagnosed and relapsed patients. However, the continuing challenges physicians are facing within specific clinical settings and patient subpopulations, whose prognosis with current strategies is extremely poor, call for a paradigm change. New immunomodulators, proteasome inhibitors, histone deacetylase inhibitors, and monoclonal antibodies are being explored to improve first-line outcomes so that a smaller proportion of patients relapse early or fail to respond to induction treatment. Moreover, recent advances and clinical evidence with novel therapies seem to provide patients with relapsed or refractory MM additional survival benefits. Improving clinical outcomes and refining standard of care should help clinicians reduce the burden of multiple and toxic therapy; quality of life (QoL) should be at the core of MM management. Patient selection and stratification needs to be reinforced with the help of comprehensive knowledge on conventional risk factors, and supplemented by molecular pathways in the near future in order to provide tailored options and strategies to patients, including the use of monoclonal antibodies. Numerous drugs are on the horizon and the next few years should witness marked improvements in survival, QoL, and safety of MM management.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117235223","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}
Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10313345
L. Varricchio, A. Migliaccio
Calreticulin (CALR), a Ca2+ binding protein mostly localised in the endoplasmic reticulum, regulates Ca2+ homeostasis, chaperones, and other proteins to the nucleus and other cellular compartments. CALR has been implicated in several cellular processes including: signalling, regulation of gene expression, cell adhesion, apoptosis, autoimmunity, and, when expressed on the cell surface, induction of phagocytosis by macrophages. Reports indicating over-expression of CALR in cancer cells suggest that modulation of CALR expression may be exploited to increase their clearance by the immune system. In the hematopoietic system, CALR has been implicated in the activation of the stress pathway as an obligatory partner of the glucocorticoid receptor. More recently, somatic loss-of-function mutations in the CALR gene were discovered in a significant proportion of patients with Philadelphia-negative myeloproliferative neoplasms (MPN) who did not harbour gain-of-function mutations in Janus kinase 2 (JAK2), the first signalling element of cytokine receptors, and myeloproliferative leukaemia virus oncogene (MPL), the thrombopoietin receptor, usually associated with these diseases. This review will summarise current knowledge on the biological activity of CALR and MPL/JAK2 in hematopoiesis, delineate a unifying pathway for the pathogenesis of MPN, and discuss how this pathway may be exploited for therapy.
{"title":"Calreticulin in Myeloproliferative Neoplasms: The Other Side of the Alice Mirror","authors":"L. Varricchio, A. Migliaccio","doi":"10.33590/emjhematol/10313345","DOIUrl":"https://doi.org/10.33590/emjhematol/10313345","url":null,"abstract":"Calreticulin (CALR), a Ca2+ binding protein mostly localised in the endoplasmic reticulum, regulates Ca2+ homeostasis, chaperones, and other proteins to the nucleus and other cellular compartments. CALR has been implicated in several cellular processes including: signalling, regulation of gene expression, cell adhesion, apoptosis, autoimmunity, and, when expressed on the cell surface, induction of phagocytosis by macrophages. Reports indicating over-expression of CALR in cancer cells suggest that modulation of CALR expression may be exploited to increase their clearance by the immune system. In the hematopoietic system, CALR has been implicated in the activation of the stress pathway as an obligatory partner of the glucocorticoid receptor. More recently, somatic loss-of-function mutations in the CALR gene were discovered in a significant proportion of patients with Philadelphia-negative myeloproliferative neoplasms (MPN) who did not harbour gain-of-function mutations in Janus kinase 2 (JAK2), the first signalling element of cytokine receptors, and myeloproliferative leukaemia virus oncogene (MPL), the thrombopoietin receptor, usually associated with these diseases. This review will summarise current knowledge on the biological activity of CALR and MPL/JAK2 in hematopoiesis, delineate a unifying pathway for the pathogenesis of MPN, and discuss how this pathway may be exploited for therapy.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130040141","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}
Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10311605
V. Popov, M. Onisâi, A. Vlădăreanu
Patients diagnosed with myeloproliferative neoplasms (MPNs) often develop thrombotic events as an onset of symptoms or in evolution. The pathogenesis of thrombosis in patients with MPN is multifactorial. There are multiple prognostic score systems, but the presence of JAK2V617F (JAK2) mutation is an independent and strong thrombosis risk factor. Patients with MPN and JAK mutational status usually associate thrombocytosis, increased immature circulating platelets, and leukocytosis, with increased expression of CD62P and CD14, increased levels of circulating microparticles and leuko-platelet microaggregates, and altered endothelial function. This review aims to discuss different factors contributing to the increased thrombotic risk in association with JAK2 mutational status. Also, recent reports incriminate this mutation to have a possible role in spontaneous loss of pregnancy.
{"title":"The Role of JAK2 Mutation in Thrombotic Complications of Chronic Myeloproliferative Neoplasms","authors":"V. Popov, M. Onisâi, A. Vlădăreanu","doi":"10.33590/emjhematol/10311605","DOIUrl":"https://doi.org/10.33590/emjhematol/10311605","url":null,"abstract":"Patients diagnosed with myeloproliferative neoplasms (MPNs) often develop thrombotic events as an onset of symptoms or in evolution. The pathogenesis of thrombosis in patients with MPN is multifactorial. There are multiple prognostic score systems, but the presence of JAK2V617F (JAK2) mutation is an independent and strong thrombosis risk factor. Patients with MPN and JAK mutational status usually associate thrombocytosis, increased immature circulating platelets, and leukocytosis, with increased expression of CD62P and CD14, increased levels of circulating microparticles and leuko-platelet microaggregates, and altered endothelial function. This review aims to discuss different factors contributing to the increased thrombotic risk in association with JAK2 mutational status. Also, recent reports incriminate this mutation to have a possible role in spontaneous loss of pregnancy.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122590295","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}
Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10313844
J. Bell
Prof Zelenetz opened the symposium on the evolving front-line treatment options for follicular lymphoma (FL) and discussed the potential of novel agents to replace chemotherapy. Prof Zelenetz presented the heterogeneity of diffuse large B cell non-Hodgkin’s lymphoma (DLBCL) with regard to the diagnosis and subtypes of DLBCL to describe the specificity of new agents towards certain DLBCL subgroups, whilst Prof Dreyling spoke about the current diagnosis and treatment pathways for mantle cell lymphoma (MCL), and briefly described recent trial results. The final presenter, Prof Coiffier, discussed the lack of efficacy of front-line chemotherapy regimens for peripheral T cell lymphoma (PTCL), and highlighted potential new treatments based upon CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone). He then addressed the use of transplantation for first-line and refractory disease, and called for research to optimise therapy using existing agents.
{"title":"Next Steps in Front-Line Treatment of Lymphoma: The Road Ahead","authors":"J. Bell","doi":"10.33590/emjhematol/10313844","DOIUrl":"https://doi.org/10.33590/emjhematol/10313844","url":null,"abstract":"Prof Zelenetz opened the symposium on the evolving front-line treatment options for follicular lymphoma (FL) and discussed the potential of novel agents to replace chemotherapy. Prof Zelenetz presented the heterogeneity of diffuse large B cell non-Hodgkin’s lymphoma (DLBCL) with regard to the diagnosis and subtypes of DLBCL to describe the specificity of new agents towards certain DLBCL subgroups, whilst Prof Dreyling spoke about the current diagnosis and treatment pathways for mantle cell lymphoma (MCL), and briefly described recent trial results. The final presenter, Prof Coiffier, discussed the lack of efficacy of front-line chemotherapy regimens for peripheral T cell lymphoma (PTCL), and highlighted potential new treatments based upon CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone). He then addressed the use of transplantation for first-line and refractory disease, and called for research to optimise therapy using existing agents.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117016320","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}
Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10310448
Saroshi Amirthalingam
The Alexion satellite symposium was introduced by Prof Pier Mannuccio Mannucci who provided an introduction to the history of thrombotic microangiopathies (TMAs). Prof Paul Coppo gave an overview of TMAs and the differential diagnosis of atypical hemolytic-uraemic syndrome (aHUS) and thrombotic thrombocytopaenic purpura (TTP). He emphasised the importance of a suitable differential diagnosis in order to initiate an appropriate treatment as soon as possible, eventually allowing better patient outcomes. Prof Javier de la Rubia discussed the role of the complement pathway and how genetic abnormalities can lead to dysregulation in aHUS. Prof Thorsten Feldkamp concluded by giving an overview of the latest clinical trial data on the efficacy and safety of eculizumab in the management of aHUS.
Alexion卫星研讨会由Pier Mannuccio Mannucci教授介绍，他介绍了血栓性微血管病变(TMAs)的历史。Paul Coppo教授概述了TMAs和非典型溶血性尿毒综合征(aHUS)和血栓性血小板减少性紫癜(TTP)的鉴别诊断。他强调了适当的鉴别诊断的重要性，以便尽快开始适当的治疗，最终使患者获得更好的结果。Javier de la Rubia教授讨论了补体途径的作用以及遗传异常如何导致aHUS的失调。Thorsten Feldkamp教授总结了eculizumab在aHUS治疗中的有效性和安全性的最新临床试验数据。
{"title":"The Multiple Facets of Thrombotic Microangiopathies","authors":"Saroshi Amirthalingam","doi":"10.33590/emjhematol/10310448","DOIUrl":"https://doi.org/10.33590/emjhematol/10310448","url":null,"abstract":"The Alexion satellite symposium was introduced by Prof Pier Mannuccio Mannucci who provided an introduction to the history of thrombotic microangiopathies (TMAs). Prof Paul Coppo gave an overview of TMAs and the differential diagnosis of atypical hemolytic-uraemic syndrome (aHUS) and thrombotic thrombocytopaenic purpura (TTP). He emphasised the importance of a suitable differential diagnosis in order to initiate an appropriate treatment as soon as possible, eventually allowing better patient outcomes. Prof Javier de la Rubia discussed the role of the complement pathway and how genetic abnormalities can lead to dysregulation in aHUS. Prof Thorsten Feldkamp concluded by giving an overview of the latest clinical trial data on the efficacy and safety of eculizumab in the management of aHUS.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115547022","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}
Pub Date : 2014-07-31DOI: 10.33590/emjhematol/10314573
M. Hutchings
CD30-positive (CD30+) lymphomas are a heterogeneous group of hematological malignancies that share the same antigen. Over recent decades, advances in therapeutic management of these diseases have considerably improved clinical outcomes. Overall, the two main CD30+ lymphomas – Hodgkin’s lymphoma and systemic anaplastic large cell lymphoma – are associated with a favourable prognosis after first-line therapy. Nevertheless, optimal therapeutic strategies are needed to manage relapsed or refractory CD30+ lymphomas. The introduction of novel targeted approaches, such as brentuximab vedotin (BV), expands the therapeutic armamentarium and provides new perspectives in terms of clinical efficacy despite heavily pretreated disease, with reasonable toxicity to patients whose quality of life is often impaired by the disease and repeated treatments. The standard of care (SoC) for these malignancies is being refined and will be clarified with results from ongoing and upcoming Phase II/III clinical trials. Clinical studies are currently assessing the use of BV in a broad range of CD30+ lymphomas. Over time, frontline strategies and SoC will be refined in order to improve outcomes for patients with relapsed disease, while allowing clinicians to expand patient selection and provide long-term remission in a wide variety of clinical settings.
{"title":"The Standard of Care in Relapsed Refractory CD30+ Lymphoma","authors":"M. Hutchings","doi":"10.33590/emjhematol/10314573","DOIUrl":"https://doi.org/10.33590/emjhematol/10314573","url":null,"abstract":"CD30-positive (CD30+) lymphomas are a heterogeneous group of hematological malignancies that share the same antigen. Over recent decades, advances in therapeutic management of these diseases have considerably improved clinical outcomes. Overall, the two main CD30+ lymphomas – Hodgkin’s lymphoma and systemic anaplastic large cell lymphoma – are associated with a favourable prognosis after first-line therapy. Nevertheless, optimal therapeutic strategies are needed to manage relapsed or refractory CD30+ lymphomas. The introduction of novel targeted approaches, such as brentuximab vedotin (BV), expands the therapeutic armamentarium and provides new perspectives in terms of clinical efficacy despite heavily pretreated disease, with reasonable toxicity to patients whose quality of life is often impaired by the disease and repeated treatments. The standard of care (SoC) for these malignancies is being refined and will be clarified with results from ongoing and upcoming Phase II/III clinical trials. Clinical studies are currently assessing the use of BV in a broad range of CD30+ lymphomas. Over time, frontline strategies and SoC will be refined in order to improve outcomes for patients with relapsed disease, while allowing clinicians to expand patient selection and provide long-term remission in a wide variety of clinical settings.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132028730","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}
Pub Date : 2014-05-27DOI: 10.33590/emjhepatol/10310152
A. Canbay, G. Gerken
Despite progress in understanding the underlying mechanisms, acute liver failure (ALF) is still one of the major clinical challenges in hepatology. A wide variety of aetiologies, and similarly, variable courses of the disease, make it crucial to identify the cause of ALF in each individual patient. Conservative therapy is only available for some patients; for many others, liver transplantation remains the only curative option for ALF. Thus, early evaluation and prognostication of the ALF syndrome is warranted for a timely decision to list a patient for transplantation or even as high urgency. This review aims to compose our current knowledge on epidemiology, mechanisms, and prognosis in ALF, and to give a perspective for future studies in this field.
{"title":"Acute Liver Failure: A Dangerous and Challenging Syndrome","authors":"A. Canbay, G. Gerken","doi":"10.33590/emjhepatol/10310152","DOIUrl":"https://doi.org/10.33590/emjhepatol/10310152","url":null,"abstract":"Despite progress in understanding the underlying mechanisms, acute liver failure (ALF) is still one of the major clinical challenges in hepatology. A wide variety of aetiologies, and similarly, variable courses of the disease, make it crucial to identify the cause of ALF in each individual patient. Conservative therapy is only available for some patients; for many others, liver transplantation remains the only curative option for ALF. Thus, early evaluation and prognostication of the ALF syndrome is warranted for a timely decision to list a patient for transplantation or even as high urgency. This review aims to compose our current knowledge on epidemiology, mechanisms, and prognosis in ALF, and to give a perspective for future studies in this field.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2014-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134086550","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}
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