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Best Practice & Research. Clinical Haematology

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https://www.readbyqxmd.com/read/29452672/what-is-the-optimal-initial-management-of-the-older-mcl-patient
#1
REVIEW
Johanna C Kluin-Nelemans, Jeanette K Doorduijn
The current first line treatment of a patient with mantle cell lymphoma (MCL) is often considered as too toxic for elderly patients. The elderly, however, comprise the majority of the patients with MCL. The results of several recent studies have shown that the outcome of this patient group is not as dismal as in the past. Indeed, if patients are not considered frail, and can tolerate rituximab and moderate intensive chemotherapy such as R-CHOP followed by rituximab maintenance or R-bendamustine, a 4-year overall survival of >80% can be achieved...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452671/what-is-the-optimal-initial-management-of-the-younger-mantle-cell-lymphoma-patient
#2
REVIEW
Rory McCulloch, Simon Rule
The last 20 years has seen considerable advances made in the management of younger patients with mantle cell lymphoma. The use of high dose cytarabine and rituximab in induction therapy, usually followed by autologous stem cell transplant consolidation, has become established practice and the median overall survival now exceeds 10 years. However, this high intensity upfront approach is not necessarily appropriate for all newly diagnosed patients. A minority exhibit disease that behaves in an indolent fashion with no proven benefit from early intervention, and at the opposite end of the spectrum a high-risk group exists who do poorly with conventional treatment...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452670/what-is-the-optimal-management-of-older-cll-patients
#3
REVIEW
Jennifer A Woyach
CLL is the most common leukemia in older adults with a median age at diagnosis of 71. Therefore, management of patients with this disease must take into account the older age of most patients and consequences of this in terms of functional status and organ function. This review will discuss the management of CLL with regards to observation prior to the initiation of therapy, functional status, and initial treatment. We will discuss criteria for the initiation of therapy, and how initial therapy is different between older and younger patients...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452669/optimal-management-of-the-young-patient-cll-patient
#4
REVIEW
John N Allan, Richard R Furman
The emergence of targeted therapy for patients with chronic lymphocytic leukemia (CLL) has permanently altered the therapeutic landscape. In both upfront and relapsed settings, safe and effective oral kinase inhibitors are available which rival the responses and durability seen with standard chemo immunotherapy regimens. In 2016, ibrutinib was granted Federal Drug Administration approval for first-line therapy in patients with CLL. While its role as initial therapy for older, unfit or deleted 17p CLL patients is less controversial, its role as first-line treatment for younger fit patients is less clear, begging the question, what is the optimal treatment for these patients, novel agents or standard CIT strategies? In this review, we aim to provide guidance for what we believe is the optimal management of young fit patients with CLL...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452668/should-rituximab-replace-splenectomy-in-the-management-of-splenic-marginal-zone-lymphoma
#5
REVIEW
Christina Kalpadakis, Gerassimos A Pangalis, Maria K Angelopoulou, Sotirios Sachanas, Theodoros P Vassilakopoulos
SMZL is a relatively rare low grade B-cell lymphoma, characterized usually by an indolent clinical behavior. Since there is no prospective randomized trials to establish the best treatment approach, decision on therapeutic management should be based on the available retrospective series. Based on these data, rituximab and splenectomy appear to be the most effective. Splenectomy represented the standard treatment modality until early 2000s. More than 90% of the patients present quick amelioration of splenomegaly related symptoms along with improvement of cytopenias related to hypersplenism...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452667/overview-on-the-management-of-non-gastric-malt-lymphomas
#6
REVIEW
Irene Defrancesco, Luca Arcaini
Extranodal marginal zone B-cell lymphomas (EMZLs) of the mucosa-associated lymphoid tissue (MALT) are indolent lymphomas which can present at any extranodal site. The most frequent localizations (other than stomach) are ocular adnexa, salivary gland, skin, lung and thyroid. Chronic inflammation and antigenic stimulation are a potential risk for the development of MALT lymphomas. While Helicobacter Pylori (HP) is known to be associated with gastric MALT lymphoma and antibiotic therapy is effective in the setting of HP-positive, other microorganisms (such as Chlamydophila Psittaci, Campylobacter Jejiuni, Borrelia Burgdoferi) have been implicated in the pathogenesis of non-gastric MALT lymphomas...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452666/transformation-of-follicular-lymphoma-why-does-it-happen-and-can-it-be-prevented
#7
REVIEW
Brian K Link
Follicular lymphoma is a clinical disease with a multitude of presentations and behaviors. Although infrequent, transformation of follicular lymphoma to a more aggressive behaving subtype - prototypically diffuse large B-cell lymphoma - confers a substantially adverse prognosis. There is no consensus for optimal management after transformation is recognized. Historically considered a distinct clinical event, this review highlights the multiple subclinical transformational events that either variably or cumulatively result in clinical recognition of transformed follicular lymphoma...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452665/novel-agents-for-relapsed-and-refractory-follicular-lymphoma
#8
REVIEW
Chan Yoon Cheah, Nathan H Fowler
Follicular lymphoma is one of the most common non-Hodgkin's lymphomas. Although current frontline regimens are associated with high response rates, most patients still relapse. When progression is discovered, re-establishing the diagnosis and ruling out transformation in paramount. The outcomes following relapse have been improving due to the activity and increasing availability of novel agents with various mechanisms of action. Despite these advances, single agent activity is limited and the disease remains incurable in the majority of cases...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452664/the-role-of-stem-cell-transplantation-in-follicular-lymphoma
#9
REVIEW
Georg Hess
With the introduction of novel treatments paradigms to if or when to use transplantation strategies for patients with follicular lymphoma have changed substantially. Autologous transplantation has been intensively evaluated as consolidation after first induction treatment with positive effects, however the introduction of Rituximab led to comparable improvements and HDT has been moved to relapse treatment. In this indication HDT was frequently use already at first relapse, but now is dominantly used in patients with a highrisk profile, e...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452663/management-of-untreated-advanced-stage-follicular-lymphoma-role-of-patient-discernment
#10
REVIEW
Jayadev Manikkam Umakanthan, Mathew A Lunning
Follicular lymphoma is the most common indolent non-Hodgkin lymphoma. Advanced stage disease is common at diagnosis. The timing of treatment for follicular lymphoma is best approached by considering the combination of presence or absence of symptoms along with estimation of tumor burden. Upfront treatment strategies should take into initial presentation variables, pace of disease progression and goals of care after discussion with the patient. Treatment approaches remain diverse and patient discernment is paramount...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452662/pathogenesis-of-follicular-lymphoma
#11
REVIEW
Tracy Lackraj, Rashmi Goswami, Robert Kridel
Follicular lymphoma (FL) is presented as a germinal centre B cell lymphoma that is characterized by an indolent clinical course, but remains - paradoxically - largely incurable to date. The last years have seen significant progress in our understanding of FL lymphomagenesis, which is a multi-step process beginning in the bone marrow with the hallmark t(14;18)(q32;q21) translocation. The pathobiology of FL is complex and combines broad somatic changes at the level of both the genome and the epigenome, the latter evidenced by highly recurrent mutations in chromatin-modifying genes such as KMT2D and CREBBP...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452661/risk-stratification-in-follicular-lymphoma
#12
REVIEW
Carla Casulo
Advances in the understanding of FL biology, molecular characteristics and clinical risk factors are further highlighting the heterogeneity of this disease. Historically used prognostic factors were broadly based on clinical and laboratory features at the time of diagnosis. However novel prognostic factors are emerging that can be studied at the time of diagnosis, and relapse, and use a variety of tools including gene alterations and diagnostic imaging. These novel discoveries are being implemented into daily practice with the ultimate goal of providing a precise, individualized approach to every patient with FL...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452660/novel-therapies-for-relapsed-refractory-mantle-cell-lymphoma
#13
REVIEW
Puja C Arora, Craig A Portell
Mantle cell lymphoma is an aggressive Non-Hodgkin's lymphoma that is considered incurable with standard therapies. Most patients treated with frontline immunochemotherapy relapse within a few years and do not usually respond to salvage chemotherapy. Persistent activation of the B-cell receptor pathway is critical to the pathogenesis of mantle cell lymphoma. Inhibition of Bruton's tyrosine kinase, an essential B-cell receptor pathway component with ibrutinib has shown clinical activity and has changed how MCL is treated in the relapsed/refractory setting...
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29452659/indolent-lymphomas
#14
EDITORIAL
Brad Kahl
No abstract text is available yet for this article.
March 2018: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29156207/intracellular-protein-degradation-from-a-vague-idea-thru-the-lysosome-and-the-ubiquitin-proteasome-system-and-onto-human-diseases-and-drug-targeting
#15
REVIEW
Aaron Ciechanover
Between the 1950s and 1980s, scientists were focusing mostly on how the genetic code is transcribed to RNA and translated to proteins, but how proteins are degraded has remained a neglected research area. With the discovery of the lysosome by Christian de Duve it was assumed that cellular proteins are degraded within this organelle. Yet, several independent lines of experimental evidence strongly suggested that intracellular proteolysis is largely non-lysosomal, but the mechanisms involved remained obscure...
December 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29156206/cytokine-release-syndrome-who-is-at-risk-and-how-to-treat
#16
REVIEW
Noelle Frey
T-cell engaging therapies such as blinatumomab and anti-CD19 chimeric antigen receptor (CAR) T cells have revolutionized our approach to patients with relapsed and refractory acute lymphoblastic leukemia (ALL). However, the immune activation responsible for high remission rates is also responsible for the unique treatment-related toxicity of cytokine release syndrome (CRS). The clinical signs of CRS include fever, hemodynamic instability, and capillary leak, which correlate with T-cell activation and elevated cytokine levels...
December 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29156205/which-factors-influence-the-development-of-gvhd-in-hla-matched-or-mismatched-transplants
#17
REVIEW
Effie W Petersdorf
The sheer diversity of HLA alleles makes the probability of finding matched unrelated donors for patients requiring hematopoietic cell transplantation (HCT) a complex situation. New evidence suggests that mismatching at certain HLA loci may provide a greater benefit in terms of graft-versus-leukemia effect than other mismatches when HLA-matched donors are not available. This review summarizes the current understanding of HLA matching requirements for unrelated donor HCT.
December 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29156204/autologous-hematopoietic-cell-transplantation-for-adult-acute-myeloid-leukemia-an-obsolete-or-resurfacing-concept
#18
REVIEW
Hillard M Lazarus, Najla El Jurdi
Improving long-term outcomes of adult acute myeloid leukemia (AML) patients remains a challenge. Major scientific and clinical advances have led to a better understanding of the disease biology, and the majority of patients achieve a complete remission (CR) after induction therapy. Relapse risk, however, remains considerable and is the leading cause of death in this patient population. Significant efforts to improve outcomes emphasize use of post-remission therapies such as hematopoietic cell transplantation (HCT), an increasingly utilized modality...
December 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29156203/impact-of-allogeneic-hematopoietic-cell-transplantation-on-the-outcome-of-older-patients-with-acute-myeloid-leukemia
#19
REVIEW
Frederick R Appelbaum
For younger patients with intermediate- or high-risk acute myeloid leukemia (AML) in first remission, allogeneic hematopoietic cell transplantation (HCT) offers the best chance of cure and therefore is the treatment of choice. The role of allogeneic HCT in the treatment of older patients is less well defined. In this review, four issues concerning the role of HCT in the treatment of older AML patients will be addressed: the frequency of allogeneic HCT in the older AML population in the US; the impact of age on the outcome of HCT; the comparative outcome of allogeneic HCT versus chemotherapy in older AML patients; and some of the barriers to the effective use of HCT in older AML patients...
December 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29156202/relapsed-acute-lymphoblastic-leukemia-is-it-crucial-to-achieve-molecular-remission-prior-to-transplant
#20
REVIEW
Mary Eapen
In patients with acute lymphoblastic leukemia (ALL) the risk of recurrent leukemia influences the choice of treatment between chemotherapy and allogeneic hematopoietic cell transplantation. The evaluation of minimal residual disease (MRD) is now considered to be the greatest progress in risk stratification in regard to leukemia recurrence. Achieving molecular remission at the end of induction therapy after diagnosis or after relapse has influenced treatment choice. Failure to achieve molecular remission is considered "high risk" and allogeneic hematopoietic cell transplantation with a suitable donor, the accepted standard...
December 2017: Best Practice & Research. Clinical Haematology
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