Best Practice & Research Clinical Haematology RSS feed: Current Issue. In practical paperback format, each 200 page topic-based issue of Best Practice & Research Clinical Haematology will provide a comprehensive review of current clinical practice and thinking within the specialty of haematology. All chapters are commissioned and written by an international team of practising clinicians with the Guest Editors for each issue drawn from a pool of renowned experts and opinion leaders. Reference is made to: • the latest original research • Cochrane Reviews • audits and confidential enquiries • national and international conferences • national and international guidelines • personal communications All chapters take the form of practical, evidence-based reviews that seek to address key clinical issues of diagnosis, treatment and patient management. Each issue follows a problem-orientated approach that focuses on the key questions to be addressed, clearly defining what is known and not known. Management will be described in practical terms so that it can be applied to the individual patient. Boxed and bulleted Learning Objectives and Practice Points are features within each chapter and will highlight the core and essential knowledge that will help the physician to provide the best care to their patients. The series' objective is to provide a continuous update for the busy clinician and researcher. http://www.bprch.com/?rss=yesElsevier Inc.en © 2012 Published by Elsevier Inc. All rights reserved. Best Practice & Research Clinical Haematology1521-6926251March 2012 © 2012 Published by Elsevier Inc. All rights reserved. healthpermissions@elsevier.comhttp://www.bprch.com/article/PIIS1521692612000163/abstract?rss=yesEditorial Board / Aims & Scope10.1016/S1521-6926(12)00016-3Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-1iiiiiihttp://www.bprch.com/article/PIIS1521692612000114/abstract?rss=yesThe management of diffuse large B-cell lymphoma (DLBCL) has completely changed since the introduction of the monoclonal antibody anti-CD20 in combination with conventional chemotherapy. Currently, more than 50% of DLBCL cases are curable, depending on age   to 60 years and prognostic factors. In this volume, the authors of this volume will focus on some of the most difficult situations for the management of DLBCL and other high grade, including T and B-cell lymphomas.PrefaceChristian Gisselbrecht10.1016/j.beha.2012.02.001Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-112http://www.bprch.com/article/PIIS1521692612000047/abstract?rss=yesDiffuse large B-cell lymphoma and Burkitt lymphoma are two distinct lymphoma entities recognized by the current World Health Organization classification of lymphoid neoplasms. Although diagnostic criteria to distinguish these two subtypes have been established, a subset of cases have overlapping characteristics and therefore, the distinction can be challenging. Thus, the World Health Organization classification introduced an intermediate subtype referred as “B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma”. This review summarizes our current understanding of the biology of these lymphoma subtypes.New insights into the biology of molecular subtypes of diffuse large B-cell lymphoma and Burkitt lymphomaMareike Frick, Bernd Dörken, Georg Lenz10.1016/j.beha.2012.01.003Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-1312http://www.bprch.com/article/PIIS1521692612000059/abstract?rss=yesPeripheral T-cell lymphomas (PTCLs) are heterogeneous and uncommon malignancies characterized by an aggressive clinical course and a mostly poor outcome with current treatment strategies. The recent genome-wide molecular characterization of several entities has provided novel insights into their pathobiology and led to the identification of new biomarkers with diagnostic, prognostic or therapeutic implications for PTCL patients. Cell lineage and differentiation antigens (markers of γδ or NK lineage, of cytotoxicity, of follicular helper T cells) reflect the tumour’s biological behaviour, and their detection in tissue samples may refine the diagnostic and prognostic stratification of the patients. Previously unrecognized gene rearrangements are being discovered (ITK-SYK translocation, IRF4/MUM1 and DUSP22 rearrangements), and may serve as diagnostic genetic markers. Deregulated molecules within oncogenic pathways (NF-κB, Syk, PDGFRα) and immunoreactive cell-surface antigens (CD30, CD52) have been brought to the fore as potential targets for guiding the development of novel therapies.New biomarkers in T-cell lymphomasBettina Bisig, Philippe Gaulard, Laurence de Leval10.1016/j.beha.2012.01.004Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-11328http://www.bprch.com/article/PIIS1521692612000072/abstract?rss=yesCore needle biopsy is increasingly replacing excisional lymph node biopsy in the diagnosis and subclassification of malignant lymphomas, with obvious advantages in terms of morbidity and costs. This technique has radically altered the diagnostic strategy of enlarged lymph nodes at our institution, avoiding unnecessary nodal excisions. It represents a viable alternative as long as the number and size of cores for morphologic and molecular studies are not compromised. This quick and safe technique can be applied to the initial diagnostic evaluation of malignant lymphomas as well as the reassessment of previously diagnosed malignant lymphomas at time of progression or recurrence.Image-guided needle biopsy for diagnosis and molecular biology in lymphomasE. de Kerviler, C. Benet, J. Brière, C. de Bazelaire10.1016/j.beha.2012.01.006Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-12939http://www.bprch.com/article/PIIS1521692612000096/abstract?rss=yesClinically this chapter will focus on 3 important issues critical in the management of diffuse large B cell lymphoma (DLBCL) in 2012: risk stratification, interim PET-CT scanning and the role of high dose therapy and ASCT in the rituximab era for patients with relapsed and primary refractory disease.Diffuse large B Cell lymphoma: How can we cure more patients in 2012?Craig Moskowitz10.1016/j.beha.2012.01.008Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-14147http://www.bprch.com/article/PIIS1521692612000035/abstract?rss=yesWe now better understand that the treatment of Diffuse Large B-cell lymphoma (DLBCL) must take into account individual factors related to the biological characteristics of tumors and patients. Treatment failure has dramatically reduced with the combination of rituximab and chemotherapy, mostly in the germinal center B (GCB) cell-like subset. However, salvage chemotherapy and autotransplantation are less effective in patients with previous exposure to rituximab. Therefore, new therapies should focus on poor-risk patients or on the non-CG subset.After reviewing the recent data on salvage strategy, we aimed to focus on novel agents that have been shown to be promising for future therapy of DLBCL (e.g., monoclonal antibody-based therapy and small-molecule inhibitors) and, lastly, to present perspectives on the use of these new agents in combination.Relapses, treatments and new drugsNicolas Mounier, Christian Gisselbrecht10.1016/j.beha.2012.01.002Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-14960http://www.bprch.com/article/PIIS1521692612000084/abstract?rss=yesThe roles of HDT/autoSCT and alloSCT in the treatment of B- and T-cell lymphomas continue to change. With the wider use of Rituximab in virtually every patient with B-cell lymphoma transplantation as part of first-line therapy has been challenged. New studies fail to show a benefit of HDT/autoSCT over conventional therapy when administered to newly diagnosed patients with aggressive B-cell lymphoma; in the other B-cell lymphomas results from randomized studies are not yet available.Patients relapsing from first-line therapy including Rituximab do not have satisfying results with HDT/autoSCT. Therefore, alloSCT is increasingly being considered.In T-cell lymphoma the efficacy of autoSCT seems rather limited. Study groups and single institutions are developing new strategies including alloSCT to improve the situation for such patients both in the setting of first-line and salvage therapy.Autologous or allogeneic transplantation in B- and T-cell lymphomasN. Schmitz, M. Nickelsen, B. Glaß10.1016/j.beha.2012.01.007Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-16173http://www.bprch.com/article/PIIS1521692612000060/abstract?rss=yesEpstein-Barr virus (EBV) is a ubiquitous γ-herpes virus that infects most people but results in life-threatening diseases in only a small subset. Persons who are unable to maintain the virus in its latent state can develop uncontrolled EBV-driven lymphoproliferative disorders and lymphomas. EBV-associated lymphomas are well characterized in patients with known defects in cellular immunity as occurs post-transplantation or HIV/AIDS but are increasingly recognized in patients without overt immunodeficiencies. Improved understanding of the biology of these lymphomas and the role EBV plays in lymphomagenesis offer the opportunity for improved therapies targeted at important signaling pathways and immunotherapy specific against EBV viral antigens.EBV-associated lymphomas in adultsMark Roschewski, Wyndham H. Wilson10.1016/j.beha.2012.01.005Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-17589http://www.bprch.com/article/PIIS1521692612000102/abstract?rss=yesManagement of the elderly with lymphoma needs specific attention. This means supplementary evaluation as regards to younger patients. The objective is to identify specific weaknesses of the patients and thus to foresee potential unexpected toxicities which may endanger patients’ outcome. With this information, the hematologist will be able to propose an optimized treatment strategy i.e. with an adapted efficacy/toxicity ratio.These general principles are consensual but it remains to determine what the practical content of this approach is. Recent results suggest some specific tools for the pre-treatment evaluation. Some specific indexes have been proposed to categorize patients but the ultimate approach remains to be outlined. Finally, while geriatric intervention has demonstrated its capacity to improve survival in the general elderly population, no such demonstration has been made in cancer patients including lymphoma.Therapeutic strategies in elderly and very elderly patientsP. Soubeyran, B. Henriques de Figueiredo, I. Soubeyran, C. Mertens, A.L. Cazeau10.1016/j.beha.2012.01.009Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-191100http://www.bprch.com/article/PIIS1521692612000023/abstract?rss=yesThe incidence of aggressive lymphoma in the setting of HIV infection is significantly increased relative to the general population. Combination antiretroviral therapy (cART) for HIV has reduced the incidence of these neoplasms and has significantly improved clinical outcome for those who do develop lymphoma and require chemotherapy. With the possible exception of those individuals with the most severe immunocompromise, patients with HIV-associated lymphoma can be treated with the same standard immuno-chemotherapy regimens used in the immunocompetent population with similar expectations for good clinical outcome. Infusional regimens like dose adjusted EPOCH-R appear to be highly effective first-line therapy and for relapsed patients high-dose chemotherapy with autologous stem cell support is well-tolerated and effective. However, it should be recognized that there are unique risks associated with management of lymphoma in this patient population. While opportunistic infections are no longer a significant cause of death, antiretroviral agents used for management of HIV infection may interact with chemotherapeutic agents and other adjunctive therapies making communication between the treating Oncologist and the patient's primary HIV treatment provider of prime importance.HIV-associated lymphomaLawrence D. Kaplan10.1016/j.beha.2012.01.001Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-1101117http://www.bprch.com/article/PIIS1521692611001101/abstract?rss=yesPrimary CNS lymphoma (PCNSL) is a rare and aggressive brain tumor with an unsatisfactory outcome. Therapeutic progress in this field is strongly conditioned by the limited biology and the molecular knowledge about this disease, which hamperizes the identification of new targeted therapies and the poor clinical conditions and performance status of patients, rendering very difficult their enrollment in prospective trials.Chemoradiation therapy is the most commonly used strategy for patients with PCNSL, which is associated with better efficacy rates, but also with high incidence of severe neurotoxicity. As a consequence, a dilemma in PCNSL treatment is the choice between strategies designed to intensify therapy to improve the cure rate, versus strategies of treatment de-escalation to avoid severe neurotoxicity. The efficacy of chemotherapy is strongly limited by the special functional and microenvironmental characteristics of the CNS, which is variably protected by the blood–brain barrier (BBB) and includes extensive chemotherapy sanctuaries where tumor cells grow undisturbed. Drugs exhibiting a good capability to cross the BBB and drugs that can be safely administered at high doses to obtain therapeutic concentrations in the CNS are the most commonly used in the treatment of PCNSL.Consolidation after chemotherapy represents the best role for radiotherapy. Since this tumor has an infiltrative nature, the whole brain should be irradiated, with increased risk of severe neurotoxicity. Some authorities are investigating in randomized trials the impact on outcome and neurotolerability of replacing consolidation radiotherapy with other strategies, like high dose chemotherapy supported by autologous stem cell transplantation. The rationale for the use of this strategy is the administration of high doses of cytostatics to achieve therapeutic concentrations in sanctuaries, CNS organs and lymphoma tissues and to overcome drug resistance mechanisms.Future therapeutic progresses in PCNSL will be based on the expansion of molecular and biological knowledge, the improvement of therapeutic efficacy and the prevention of iatrogenic neurotoxicity.Primary CNS lymphomaAndrés J.M. Ferreri, Emerenziana Marturano10.1016/j.beha.2011.12.001Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-1119130http://www.bprch.com/article/PIIS1521692612000205/abstract?rss=yesIndex10.1016/S1521-6926(12)00020-5Best Practice & Research Clinical Haematology 25, 1 (2012)2012-03-01Best Practice & Research Clinical Haematology2012-03-01251S1521-6926(12)X0002-1II