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Hematology Articles

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225 papers 100 to 500 followers
By Terrance Comeau SCT Director
Olga K Weinberg, Aliyah R Sohani, Parul Bhargava, Valentina Nardi
Acute myeloid leukemia (AML) is characterized by a clonal expansion of undifferentiated myeloid precursors resulting in impaired hematopoiesis and bone marrow failure. In 2016, the World Health Organization (WHO) published revisions to the classification of myeloid neoplasms and acute leukemias. Similar to the 2008 classification, the updated classification incorporates clinical features, morphology, immunophenotyping, and cytogenetics, with greater emphasis on molecular genetics, to define disease entities...
March 2017: American Journal of Hematology
Francesca Palandri, Giuseppe Auteri, Michele Baccarani
Myelofibrosis (MF) is the most severe among the classical Philadelphia-negative myeloproliferative neoplasms that also include essential thrombocytemia and polycythemia vera. Myelofibrosis is characterized by numerous genetic lesions, often variously associated with each other, and by an aggressive clinical phenotype leading to severely reduced survival. Also, the inflammatory microenvironment plays a key role in disease initiation and progression. Because of the complexity of its pathogenesis and the variability of clinical features, MF is a disease that requires a personalized approach and remains orphan of curative treatments besides allogeneic transplantation...
June 2017: Hematological Oncology
Alexandra Schifferli, Thomas Kühne
In 2008, new drugs that mimic the effects of thrombopoietin became available for the treatment of primary immune thrombocytopenia, eg, romiplostim and eltrombopag. These drugs activate the thrombopoietin receptor, stimulate the production of megakaryocytes, and increase the production of platelets. Important clinical observation has been gained, such as unexpected long-term remission after stopping thrombopoietin receptor agonists. The pathophysiology of this unforeseen cure is currently the subject of discussion and is investigated in clinical trials and laboratory research projects...
April 2016: Seminars in Hematology
Stephan Mathas, Sylvia Hartmann, Ralf Küppers
The Hodgkin and Reed-Sternberg (HRS) tumor cells of classical Hodgkin lymphoma (HL), as well as the lymphocyte predominant (LP) cells of nodular lymphocyte predominant HL (NLPHL), are derived from mature B cells. However, HRS cells have largely lost their B-cell phenotype and show a very unusual expression of many markers of other hematopoietic cell lineages, which aids in the differential diagnosis between classical HL (cHL) and NLPHL and distinguishes cHL from all other hematopoietic malignancies. The bi- or multinucleated Reed-Sternberg cells most likely derive from the mononuclear Hodgkin cells through a process of incomplete cytokinesis...
July 2016: Seminars in Hematology
Andrea Gallamini, Martin Hutchings, Safaa Ramadan
In the present chapter the authors present a brief overview of the diagnostic methods proposed over time for Hodgkin lymphoma (HL) spread detection, moving from surgical procedures, through standard radiological and functional imaging techniques to the present state of the art for HL staging. The main body of the review will be dedicated to the recently published guidelines for lymphoma staging (including HL) agreed by the experts during the 12th International Congress for Malignant Lymphoma in Lugano. The recommendations of the panel on how to integrate flurodeoxyglucose positron emission tomography (FDG-PET) scan in the armamentarium of staging procedures will be presented and commented, with a special emphasis on the utility of special procedures, such as bone marrow trephine biopsy, which is deemed no longer needed in the PET era...
July 2016: Seminars in Hematology
Paul J Bröckelmann, Maria K Angelopoulou, Theodoros P Vassilakopoulos
During the last decades, the prognosis of Hodgkin lymphoma (HL) has been improved significantly with the introduction of effective chemotherapy and the implementation of risk-adapted treatment approaches. Identification of reliable risk factors is crucial to guide treatment over the course of disease. Both clinical and biological factors have been implicated in the prognosis of HL and are often used in prognostic scores to discriminate risk groups. To prevent under- or overtreatment, patients are usually assigned to one of the three widely established risk groups for first-line treatment, based solely on clinical risk factors...
July 2016: Seminars in Hematology
Andreas Engert, John Raemaekers
Hodgkin lymphoma (HL) has become one of the best curable malignancies today. This is particularly true for patients with early-stage disease. Today, most patients in this risk group are treated with a combination of chemotherapy followed by small-field radiotherapy. More recent clinical trials such as the German Hodgkin Study Group (GHSG) HD10 study demonstrated, that even two cycles of ABVD followed by 20 Gy involved-field radiation therapy (IF-RT) are sufficient and result in more than 90% of patients being cured...
July 2016: Seminars in Hematology
Theodoros P Vassilakopoulos, Peter W M Johnson
There is now good evidence that the escalated BEACOPP regimen (bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine, prednisone) is more effective in controlling advanced-stage Hodgkin lymphoma (HL) than the widely used ABVD regimen (adriamycin, bleomycin, vinblastine, dacarbazine), but the extra efficacy comes at the expense of both short- and long-term toxicity, and there is debate as to whether overall survival is affected. Baseline prognostic factors have proven of limited utility for determining which patients require more intensive therapy and recent studies have sought to use interim fluoro-deoxyglucose positron emission tomography (FDG-PET) evaluation as a means to guide the modulation of treatment, both upwards and downwards in intensity...
July 2016: Seminars in Hematology
Bastian von Tresckow, Craig H Moskowitz
Despite the high first-line cure rates in patients with Hodgkin Lymphoma (HL) still 10%-20% of patients suffer from relapsed or refractory disease. High-dose chemotherapy (HDCT) followed by autologous stem cell transplant (ASCT) is standard of care for suitable patients with relapsed or refractory HL and allows for cure in approximately 50%. Due to the poor prognosis of high-risk patients even with HDCT and ASCT, consolidation strategies have been evaluated to improve the cure rates. For patients with recurrence after HDCT and ASCT, treatment is palliative in most cases...
July 2016: Seminars in Hematology
Anas Younes, Stephen M Ansell
Hodgkin Lymphoma (HL) is a lymphoproliferative disorder of B cells that commonly has a favorable prognosis when treated with either combination chemotherapy and radiation therapy, or chemotherapy alone. However, the prognosis for patients who relapse, or have evidence for refractory disease, is poor and new treatments are needed for patients with progressive disease. HL has a unique tumor microenvironment consisting of a predominance of inflammatory cells and a minority of malignant Hodgkin and Reed-Sternberg (HRS) cells...
July 2016: Seminars in Hematology
Kerry J Savage, Anja Mottok, Michelle Fanale
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subtype of Hodgkin lymphoma with distinct clinicopathologic features. It is typified by the presence of lymphocyte predominant (LP) cells, which are CD20(+) but CD15(-) and CD30(-) and are found scattered amongst small B lymphocytes arranged in a nodular pattern. Despite frequent and often late or multiple relapses, the prognosis of NLPHL is very favorable. There is an inherent risk of secondary aggressive non-Hodgkin lymphoma (NHL) and studies support that risk is highest in those with splenic involvement at presentation...
July 2016: Seminars in Hematology
Veronika Bachanova, Joseph M Connors
Hodgkin lymphoma (HL) presenting in patients with co-incidental advanced age, pregnancy, or human immunodeficiency virus (HIV) infection is uniquely challenging to manage. In this article we integrate recent evidence and clinical expertise to present recommendations for diagnosis and therapeutic management. Older patients with HL need to be carefully evaluated for comorbidies after which judicious choice of chemotherapy should minimize functional compromise. A pregnant patient with concurrent HL should be staged with minimal use of imaging requiring ionizing radiation and treated in an individualized manner optimally combining the strategies of treatment deferral when appropriate, use of single-agent vinblastine for symptomatic disease and reservation of multi-agent chemotherapy for the small minority of patients with aggressive clinical presentation...
July 2016: Seminars in Hematology
Andrea K Ng, Flora E van Leeuwen
Long-term survivors of Hodgkin lymphoma (HL) are at risk for a range of late effects, with second malignant neoplasm and cardiovascular diseases being the leading causes of death in these patients. The excess risks remain significantly elevated decades after treatment, and are clearly associated with extent of treatment exposures. Other late effects have also been identified, such as pulmonary dysfunction, endocrinopathies, muscle atrophy, and persistent fatigue. Systemic documentation of late effects and recognition of treatment- and patient-related risk factors are important, as they inform optimal surveillance and risk-reduction strategies, as well as guide therapeutic modifications in newly diagnosed patients to minimize treatment-related complications...
July 2016: Seminars in Hematology
Scott C Howard, John McCormick, Ching-Hon Pui, Randall K Buddington, R Donald Harvey
: High-dose methotrexate (HDMTX), defined as a dose higher than 500 mg/m2 , is used to treat a range of adult and childhood cancers. Although HDMTX is safely administered to most patients, it can cause significant toxicity, including acute kidney injury (AKI) in 2%-12% of patients. Nephrotoxicity results from crystallization of methotrexate in the renal tubular lumen, leading to tubular toxicity. AKI and other toxicities of high-dose methotrexate can lead to significant morbidity, treatment delays, and diminished renal function...
December 2016: Oncologist
Prithviraj Bose, Srdan Verstovsek
Currently, prognostication in primary myelofibrosis (PMF) relies on the International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS), and DIPSS-plus, which incorporate age, blood counts, constitutional symptoms, circulating blasts, red cell transfusion need, and karyotype. Although the JAK2 V617F mutation was discovered a decade ago and MPL mutations shortly thereafter, it was the recent discovery of CALR mutations in the vast majority of JAK2/MPL-unmutated patients and recognition of the powerful impact of CALR mutations and triple-negative (JAK2/MPL/CALR-negative) status on outcome that set the stage for revision of traditional prognostic models to include molecular information...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
Carlos Besses, Alberto Alvarez-Larrán
Polycythemia vera and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms associated with thrombotic or hemorrhagic complications, and increased risk of transformation to myelofibrosis and acute myeloid leukemia. The main goal of therapy is aimed at preventing vascular events that are the leading cause of morbidity and mortality in these patients. Accordingly, risk stratification is the basis for deciding when to treat a patient with cytoreductive therapy. The European LeukemiaNet has developed a series of management recommendations for front-line and second-line therapy to provide the optimal treatment for the individual patient...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
John Mascarenhas
Myeloproliferative neoplasms (MPN) in chronic phase that evolve into blast phase (BP) hold a dismal prognosis and represent an urgent unmet clinical need. The mutational landscape of MPN-BP is distinct from de novo acute myeloid leukemia and offers insight into molecular mechanisms contributing to clonal evolution providing potential novel drug targets. A number of retrospective studies have identified patient- and disease-specific variables associated with increased risk of leukemic transformation (LT) of an underlying MPN...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
Serdar Sivgin, Mehmet Fatih Karamustafaoglu, Esra Yildizhan, Gokmen Zararsiz, Leylagul Kaynar, Bulent Eser, Mustafa Cetin, Ali Unal
INTRODUCTION: Hematopoietic stem cell transplantation is a common and preferred treatment of lymphomas in many centers. Our goal was to determine the association between pretransplant iron overload and survival in patients who underwent autologous hematopoietic stem cell transplantation (autoHSCT). PATIENTS AND METHODS: A total of 165 patients with lymphoma, who underwent autoHSCT between the years of 2007 and 2014, were included in this study. Ferritin levels were used to determine iron status; the cut-off value was 500 ng/mL...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
Stephanie A Folan, Amber Rexwinkle, Jane Autry, Jeffrey C Bryan
Adult patients with acute lymphoblastic leukemia who relapse after frontline therapy have extremely poor outcomes despite advances in chemotherapy and hematopoietic stem cell transplantation. Blinatumomab is a first-in-class bispecific T-cell engager that links T cells to tumor cells leading to T-cell activation and tumor cell lysis. In December 2014, the Food and Drug Administration approved blinatumomab for treatment of relapsed or refractory Philadelphia chromosome-negative precursor B-cell acute lymphoblastic leukemia...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
Nicholas J Short, Farhad Ravandi
Dose intensification of chemotherapy and the combination of a third cytotoxic agent with standard cytarabine and anthracycline-based induction chemotherapy have led to improved outcomes in select groups of patients with acute myeloid leukemia (AML). However, despite some progress in this area, it appears that we might be reaching the limit of cytotoxic chemotherapy for the treatment of AML, especially in older patients and in those with poor-risk features whose disease tends to be relatively chemoresistant...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
2016-08-20 10:57:36
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