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Therapeutic Advances in Hematology

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https://www.readbyqxmd.com/read/28811872/current-and-emerging-treatment-options-for-mantle-cell-lymphoma
#1
REVIEW
Bita Fakhri, Brad Kahl
Mantle cell lymphoma (MCL) is a B-cell non-Hodgkin lymphoma with typically aggressive behavior. The genetic signature is the chromosomal translocation t(11;14)(q13;q32) resulting in overexpression of cyclin D1. Asymptomatic newly diagnosed MCL patients with low tumor burden can be closely observed, deferring therapy to the time of disease progression. Although MCL classically responds to upfront chemotherapy, it remains incurable with standard approaches. For patients in need of frontline therapy, the initial decision is whether to proceed with an intensive treatment strategy or a non-intensive treatment strategy...
August 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28567239/bendamustine-and-its-role-in-the-treatment-of-unfit-patients-with-chronic-lymphocytic-leukaemia-a-perspective-review
#2
REVIEW
Othman Al-Sawaf, Paula Cramer, Valentin Goede, Michael Hallek, Natali Pflug
With a median age of 72 years at first diagnosis, chronic lymphocytic leukaemia (CLL) is a disease of the elderly. At this age, many patients cannot bear an intensive chemoimmunotherapy like fludarabine, cyclophosphamide and rituximab (FCR), and therapeutic decisions are commonly complicated by a high burden of accompanying comorbidities. Clinical trials, on the other hand, are mostly designed to include a far healthier and younger trial population, with a median age in most studies well below 70 years, leading to an insufficient reflection of clinical reality...
June 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28567238/vosaroxin-in-relapsed-refractory-acute-myeloid-leukemia-efficacy-and-safety-in-the-context-of-the-current-treatment-landscape
#3
REVIEW
Valeriy Sedov, Robert K Stuart
Treatment for acute myeloid leukemia (AML) generally consists of a combination of cytarabine and an anthracycline. Although induction therapy leads to complete remission (CR) for most patients, refractoriness to chemotherapy or relapse after initial response is associated with poor outcomes. The 1-year survival rates after first relapse have been reported at 29%, declining to 11% at 5 years. Prognosis is particularly poor among older patients whose higher prevalence of unfavorable cytogenetics and high frequency of comorbidities diminish their ability to tolerate intensive chemotherapy...
June 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28473905/targeting-the-programmed-cell-death-1-pathway-in-hodgkin-lymphoma-the-place-of-nivolumab
#4
REVIEW
Nathan D Gay, Craig Y Okada, Andy I Chen, Emma C Scott
Nivolumab is a humanized immunoglobulin gamma-4 kappa anti-programmed cell death 1 monoclonal antibody that is currently approved in the treatment of several solid tumors and recently gained accelerated approval in classical Hodgkin lymphoma (cHL) that has relapsed or progressed following autologous hematopoietic stem-cell transplantation and post-transplantation brentuximab vedotin. The purpose of this article is to review the immunophysiologic basis, clinical efficacy, and toxicity of nivolumab in the treatment of cHL...
May 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28473904/from-chronic-immune-thrombocytopenia-to-severe-aplastic-anemia-recent-insights-into-the-evolution-of-eltrombopag
#5
REVIEW
Harinder Gill, Raymond S M Wong, Yok-Lam Kwong
Thrombopoietin (TPO) is the most potent cytokine stimulating thrombopoiesis. Therapy with exogenous TPO is limited by the formation of antibodies cross-reacting with endogenous TPO. Mimetics of TPO are compounds with no antigenic similarity to TPO. Eltrombopag is an orally-active nonpeptide small molecule that binds to the transmembrane portion of the TPO receptor MPL. Initial trials of eltrombopag have centered on immune thrombocytopenia (ITP), which is due to both increased destruction and decreased production of platelets...
May 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28491266/point-of-care-ultrasonography-pocus-in-hemophilia-a-a-commentary-on-current-status-and-its-potential-role-for-improving-prophylaxis-management-in-severe-hemophilia-a
#6
REVIEW
Suchitra S Acharya, Brittny Rule, Omar McMillan, Thomas J Humphries
In patients with severe hemophilia A, recurrent bleeding into joints results in increased morbidity and reduced quality of life. Prophylaxis using replacement factor products, especially when initiated early, has established benefits in terms of reducing joint bleeds and preserving joint function. Poor adherence to prophylactic regimens is a common cause for breakthrough bleeds and resultant arthropathy. Improving prophylaxis management, especially in the transitional age group, is a challenge. Here, we discuss the current status of ultrasonography (US) in hemophilia A, challenges in its wider implementation, and the potential for use of point-of-care US (POCUS) as an adjunct in the routine management of patients with hemophilia following prophylaxis regimens...
April 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28491265/experience-with-ruxolitinib-in-the-treatment-of-polycythaemia-vera
#7
REVIEW
Samah Alimam, Claire Harrison
Polycythaemia vera (PV) is a myeloproliferative neoplasm classically characterized by an erythrocytosis and is associated with a high risk of thromboembolic events, constitutional symptoms burden and risk of transformation to myelofibrosis and acute myeloid leukaemia. Therapy is directed at the haematocrit (HCT) to reduce the risk of thrombotic events and usually comprises low-dose aspirin and phlebotomy to maintain HCT at >45%. Frequently in addition, cytoreductive therapy is indicated in high-risk patients for normalizing haematological parameters to mitigate the occurrence of thromboembolic events...
April 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28491264/priming-radioimmunotherapy-with-external-beam-radiation-in-patients-with-relapsed-low-grade-non-hodgkin-lymphoma
#8
Yazan Abuodeh, Kamran Ahmed, Michelle Echevarria, Arash Naghavi, G Daniel Grass, Timothy J Robinson, Michael Tomblyn, Bijal Shah, Julio Chavez, Celeste Bello, Ghassan El-Haddad, Louis Harrison, Sungjune Kim
BACKGROUND: The aim of this study was to evaluate the outcomes of priming salvage radioimmunotherapy (RIT) with a low dose of external beam radiotherapy (EBRT) in patients with relapsed low grade non-Hodgkin lymphoma (LG-NHL). METHODS: Patients who received salvage RIT with or without 2 × 2 Gy EBRT between March 2009 and February 2013 were retrospectively reviewed at a single institution. Planning target volume (PTV) for EBRT was created by adding a 1-2 cm expansion to the gross tumor volume depending on the anatomical location...
April 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28246555/management-of-thrombosis-in-paroxysmal-nocturnal-hemoglobinuria-a-clinician-s-guide
#9
REVIEW
Morag Griffin, Talha Munir
Paroxysmal nocturnal haemoglobinuria (PNH), an ultra-orphan disease with a prevalence of 15.9 per million in Europe, is a life-threatening disorder, characterized by haemolysis, bone marrow failure and thrombosis. Patients with PNH prior to the availability of eculizumab had a median survival of between 10 and 22 years, with thrombosis accounting for 22-67% of deaths. 29-44% of patients had at least one thrombosis. This paper provides a clinician's guide to the diagnosis, management and complications of PNH, with an emphasis on thrombosis...
March 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28246554/splanchnic-vein-thrombosis-in-myeloproliferative-neoplasms-pathophysiology-and-molecular-mechanisms-of-disease
#10
REVIEW
Joan How, Amy Zhou, Stephen T Oh
Myeloproliferative neoplasms (MPNs) are the most common underlying prothrombotic disorder found in patients with splanchnic vein thrombosis (SVT). Clinical risk factors for MPN-associated SVTs include younger age, female sex, concomitant hypercoagulable disorders, and the JAK2 V617F mutation. These risk factors are distinct from those associated with arterial or deep venous thrombosis (DVT) in MPN patients, suggesting disparate disease mechanisms. The pathophysiology of SVT is thought to derive from local interactions between activated blood cells and the unique splanchnic endothelial environment...
March 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28246553/the-role-of-combined-fludarabine-cyclophosphamide-and-rituximab-chemoimmunotherapy-in-chronic-lymphocytic-leukemia-current-evidence-and-controversies
#11
REVIEW
Alan P Skarbnik, Stefan Faderl
Chemoimmunotherapy (CIT) has become a cornerstone in the treatment of patients with chronic lymphocytic leukemia (CLL). The combination of fludarabine, cyclophosphamide and rituximab (FCR) has emerged as the standard of care for therapy of previously untreated patients with CLL who are younger than 65 years and have no significant comorbidities. In this article, we review the role of FCR in the current treatment paradigm for CLL.
March 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28203345/sweet-s-syndrome-associated-with-clonal-hematopoiesis-of-indeterminate-potential-responsive-to-5-azacitidine
#12
REVIEW
George Yaghmour, Eric Wiedower, Bassam Yaghmour, Sara Nunnery, Eric Duncavage, Mike G Martin
Sweet's syndrome (SS) is a rare condition characterized by the abrupt appearance of painful skin lesions due to neutrophilic dermal infiltration. Hematologic neoplasms, particularly acute myeloid leukemia (AML) and myelodysplastic syndromes (MDSs), have been commonly reported in association with SS. Clonal hematopoiesis of indeterminate potential (CHIP) is an emerging entity that is a precursor state to myeloid neoplasms. CHIP has not been previously associated with SS. We report the case of a 71-year-old man who presented with recurrent, painful edematous and erythematous papules and nodules for 18 months despite treatment with corticosteroids...
February 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28203344/emerging-role-of-checkpoint-blockade-therapy-in-lymphoma
#13
REVIEW
Natalie Galanina, Justin Kline, Michael R Bishop
Following the successful application of immune checkpoint blockade therapy (CBT) in refractory solid tumors, it has recently gained momentum as a promising modality in the treatment of relapsed lymphoma. This significant therapeutic advance stems from decades of research that elucidated the role of immune regulation pathways and the mechanisms by which tumors can engage these critical pathways to escape immune detection. To date, two main pathways, the cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) and programmed death 1 (PD-1), have emerged as key targets of CBT demonstrating unprecedented activity particularly in heavily pretreated relapsed/refractory Hodgkin lymphoma and some forms of non-Hodgkin disease...
February 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28203343/maintenance-therapy-in-multiple-myeloma
#14
REVIEW
Prerna Mewawalla, Abhishek Chilkulwar
Despite recent advances, multiple myeloma remains an incurable disease. Induction therapy followed by autologous transplantation has become the standard of care. The idea of maintenance therapy in multiple myeloma is not new. Starting with chemotherapy in 1975, to interferon in 1998, to novel agents recently, a multitude of agents have been explored in patients with multiple myeloma. In spite of the novel agents, multiple myeloma continues to be an incurable disease with the progression-free survival after autologous transplant rarely exceeding 3 years...
February 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28203342/optimizing-current-and-emerging-therapies-in-multiple-myeloma-a-guide-for-the-hematologist
#15
REVIEW
Shahzad Raza, Rachael A Safyan, Evan Rosenbaum, Alex S Bowman, Suzanne Lentzsch
Multiple myeloma (MM) is the second most common hematologic malignancy. The diagnosis of MM requires ⩾10% clonal plasma cells in the bone marrow or biopsy-proven plasmacytoma, plus evidence of end-organ damage (hypercalcemia, renal failure, anemia, and lytic bone lesions). The definition of MM has recently been expanded to include a ⩾60% clonal plasma cell burden in the bone marrow, serum involved/uninvolved light chain ratio of ⩾100, or more than one focal lesion on magnetic resonance imaging ⩾5 mm in the absence of end-organ damage...
February 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28203341/new-monoclonal-antibodies-on-the-horizon-in-multiple-myeloma
#16
REVIEW
Elizabeth K O'Donnell, Noopur S Raje
Across all cancers, monoclonal antibodies have emerged as a potential strategy for cancer therapy. Monoclonal antibodies target antigens expressed on the surface of cancer cells and accessory cells. This targeted approach uses the host's immune system to promote the killing of cancer cells. Multiple myeloma (MM) is the second most common hematologic malignancy that remains incurable in the majority of patients. The treatment of MM has evolved dramatically over the past decade and continues to evolve with the approval of four new drugs in 2015...
February 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28042457/an-update-on-the-role-of-daratumumab-in-the-treatment-of-multiple-myeloma
#17
REVIEW
Caitlin Costello
Monoclonal antibodies (mAbs) have emerged as a promising new drug class for the treatment of multiple myeloma (MM). Daratumumab (DARA), a CD38 mAb, has demonstrated safety, tolerability and activity in a range of clinical trials, both as monotherapy and in combination strategies for MM. The favorable efficacy results in heavily pretreated patients with advanced MM have provided the rationale for the investigation of DARA in a number of ongoing and future phase II and III trials. The general tolerability of mAbs has allowed for widespread investigation and use of DARA among a variety of MM patients, however their use requires special consideration...
January 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28042456/safety-and-efficacy-of-azacitidine-in-elderly-patients-with-intermediate-to-high-risk-myelodysplastic-syndromes
#18
REVIEW
Shyamala C Navada, Lewis R Silverman
Myelodysplastic syndromes (MDS) represent a clonal hematopoietic stem cell disorder characterized by morphologic features of dyspoiesis, a hyperproliferative bone marrow, and one or more peripheral blood cytopenias. In patients classified according to the Revised International Prognostic Scoring System (R-IPSS) with intermediate or higher-risk disease, there is an increased risk of death due to progressive bone marrow failure or transformation to acute myeloid leukemia (AML). Azacitidine was the first DNA hypomethylating agent approved by the United States (US) Food and Drug Administration (FDA) for the treatment of MDS and the only therapy that has demonstrated a significant survival benefit over conventional care regimens (CCRs) in patients with intermediate or higher-risk disease...
January 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28042455/differences-in-outcome-of-patients-with-syncytial-variant-hodgkin-lymphoma-compared-with-typical-nodular-sclerosis-hodgkin-lymphoma
#19
Tarsheen Sethi, Van Nguyen, Shaoying Li, David Morgan, John Greer, Nishitha Reddy
BACKGROUND: Nodular sclerosis Hodgkin lymphoma (NS-HL) is the most common subtype of HL and usually has a good prognosis. A variant of NS, the syncytial variant (SV) has well-established histopathologic features but little is known about its clinical behavior. Small case series have suggested that SV patients present with advanced disease and have a comparatively aggressive course. The objective of this study was to determine the clinical characteristics and outcome of SV patients. METHODS: A total of 167 adult patients with NS-HL including 43 patients with SV and 124 patients with typical NS (t-NS) were included in our analysis following institutional review board (IRB) approval...
January 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28042454/exploratory-study-on-the-impact-of-switching-to-nilotinib-in-18-patients-with-chronic-myeloid-leukemia-in-chronic-phase-with-suboptimal-response-to-imatinib
#20
Sikander Ailawadhi, Luke P Akard, Carole B Miller, Anand Jillella, Daniel J DeAngelo, Solveig G Ericson, Felice Lin, Ghulam Warsi, Jerald Radich
BACKGROUND: The phase II, exploratory, open-label Exploring Nilotinib BCR-ABL Effects (ENABL) study [ClinicalTrials.gov identifier: NCT00644878] assessed the impact of switching to nilotinib therapy in patients with chronic myeloid leukemia in chronic phase (CML-CP) who had a suboptimal molecular response with imatinib. METHODS: Patients with CML-CP who had previously achieved a complete cytogenetic response (CCyR), but had a suboptimal molecular response, with frontline imatinib therapy (N = 18) were assigned to receive nilotinib 300 mg twice daily...
January 2017: Therapeutic Advances in Hematology
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