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The absolute percent deviation of IGHV mutation rather than a 98% cut ‐off predicts survival of chronic lymphocytic leukaemia patients treated with fludarabine, cyclophosphamide and rituximab
(Source: British Journal of Haematology)
Source: British Journal of Haematology - December 3, 2017 Category: Hematology Authors: Christopher D. Gocke, Douglas E. Gladstone Tags: Editorial Comment Source Type: research

LSD1 (KDM1A) ‐independent effects of the LSD1 inhibitor SP2509 in cancer cells
(Source: British Journal of Haematology)
Source: British Journal of Haematology - December 3, 2017 Category: Hematology Authors: J ürgen Sonnemann, Miriam Zimmermann, Christian Marx, Franziska Ebert, Sabine Becker, Marie‐Luise Lauterjung, James F. Beck Tags: Correspondence Source Type: research

CD34+ selected stem cell boosts can improve poor graft function after paediatric allogeneic stem cell transplantation
We report 50 paediatric patients with PGF who received 61 boosts with CD34+ selected peripheral blood stem cells (PBSC) after transplantation from matched unrelated (n = 25) or mismatched related (n = 25) donors. Within 8 weeks, a significant increase in median neutrophil counts (0·6 vs. 1·516 × 109/l, P 
Source: British Journal of Haematology - December 3, 2017 Category: Hematology Authors: Chiara Mainardi, Martin Ebinger, Sigrid Enkel, Tobias Feuchtinger, Heiko ‐Manuel Teltschik, Matthias Eyrich, Michael Schumm, Armin Rabsteyn, Patrick Schlegel, Christian Seitz, Carl‐Phillip Schwarze, Ingo Müller, Johann Greil, Peter Bader, Paul‐Gerh Tags: research paper Source Type: research

Imatinib ‐related interstitial lung disease
(Source: British Journal of Haematology)
Source: British Journal of Haematology - December 3, 2017 Category: Hematology Authors: Zeliha Guzelkucuk, Melek Is ık, Ahmet S. Ozcan, Tulin H. Demirkan, Nese Yaralı Tags: Images in Haematology Source Type: research

Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence from the STER registry
Summary Perioperative bleeding is a major concern in patients with factor VII (FVII) deficiency. Evaluating data of 95 FVII‐deficient patients undergoing 110 surgical procedures (61 major, 49 minor), we assessed the impact of type of surgery, bleeding phenotype and FVII coagulant activity (FVII:C) levels on perioperative replacement therapy (RT). Compared to those with higher FVII:C levels, patients with
Source: British Journal of Haematology - December 1, 2017 Category: Hematology Authors: Matteo N. D. Di Minno, Mariasanta Napolitano, Alberto Dolce, Guglielmo Mariani, Tags: Research paper Source Type: research

A retrospective study on the management of patients with rituximab refractory follicular lymphoma
Summary Given that there are currently no clear recommendations regarding therapeutic options for rituximab refractory/relapsed follicular lymphoma patients, this study aimed to describe the real‐life management of patients with refractory follicular lymphoma after systemic rituximab‐containing regimens (rFL), and rFL patient characteristics. In this retrospective, national, multicentre study, descriptive analyses were mainly performed according to rituximab‐containing regimen at rFL diagnosis [rituximab monotherapy (R‐MONO), rituximab + chemotherapy (R‐COMBO), and ongoing rituximab maintenance (R‐MAI...
Source: British Journal of Haematology - December 1, 2017 Category: Hematology Authors: Philippe Solal ‐Céligny, Pierre Leconte, Aurélie Bardet, Juana Hernandez, Xavier Troussard Tags: Research Paper Source Type: research

Clinical and genetic factors are associated with pain and hospitalisation rates in sickle cell anaemia in Cameroon
Summary We aimed to investigate the clinical and genetic predictors of painful vaso‐occlusive crises (VOC) in sickle cell disease (SCD) in Cameroon. Socio‐demographics, clinical variables/events and haematological indices were acquired. Genotyping was performed for 40 variants in 17 pain‐related genes, three fetal haemoglobin (HbF)‐promoting loci, two kidney dysfunctions‐related genes, and HBA1/HBA2 genes. Statistical models using regression frameworks were performed in R®. A total of 436 hydoxycarbamide‐ and opioid‐naïve patients were studied; median age was 16 years. Female sex, body mass inde...
Source: British Journal of Haematology - December 1, 2017 Category: Hematology Authors: Ambroise Wonkam, Khuthala Mnika, Valentina J. Ngo Bitoungui, Bernard Chetcha Chemegni, Emile R. Chimusa, Collet Dandara, Andre P. Kengne Tags: Research Paper Source Type: research

A risk score including microdeletions improves relapse prediction for standard and medium risk precursor B ‐cell acute lymphoblastic leukaemia in children
Summary To prevent relapse, high risk paediatric acute lymphoblastic leukaemia (ALL) is treated very intensively. However, most patients who eventually relapse have standard or medium risk ALL with low minimal residual disease (MRD) levels. We analysed recurrent microdeletions and other clinical prognostic factors in a cohort of 475 uniformly treated non‐high risk precursor B‐cell ALL patients with the aim of better predicting relapse and refining risk stratification. Lower relapse‐free survival at 7 years (RFS) was associated with IKZF1 intragenic deletions (P 
Source: British Journal of Haematology - December 1, 2017 Category: Hematology Authors: Rosemary Sutton, Nicola C. Venn, Tamara Law, Judith M. Boer, Toby N. Trahair, Anthea Ng, Monique L. Den Boer, Anuruddhika Dissanayake, Jodie E. Giles, Pauline Dalzell, Chelsea Mayoh, Draga Barbaric, Tamas Revesz, Frank Alvaro, Rob Pieters, Michelle Haber, Tags: Research Paper Source Type: research

Advances in targeted therapy for acute myeloid leukaemia
Summary In the past few years, research in the underlying pathogenic mechanisms of acute myeloid leukaemia (AML) has led to remarkable advances in our understanding of the disease. Cytogenetic and molecular aberrations are the most important factors in determining response to chemotherapy as well as long‐term outcome, but beyond prognostication are potential therapeutic targets. Our increased understanding of the pathogenesis of AML, facilitated by next‐generation sequencing, has spurred the development of new compounds in the treatment of AML, particularly the creation of small molecules that target the disease on a m...
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Sabine Kayser, Mark J. Levis Tags: Review Source Type: research

Abdominal computed tomography angiography in post ‐transplantation sinusoidal obstruction syndrome associated with R‐DHAOX/BEAM toxicity
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Julie Lavol é, Marielle Le Goff, Amar Oudjit, Philippe Sogni, Vincent Mallet Tags: Images in Haematology Source Type: research

“Mouse‐MRD” in central nervous system acute lymphoblastic leukaemia: assessing bone marrow minimal residual disease using a xenograft model – from bedside to the bench and back again
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Julia Alten, Alexander Claviez, Simon Vieth, Gunnar Cario, Denis M. Schewe Tags: Correspondence Source Type: research

The usefulness of mutational data on prognosis of myelodysplastic syndromes: alone or incorporated into the IPSS ‐R?
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Bing Li, Jinqin Liu, Zefeng Xu, Tiejun Qin, Zhongxun Shi, Zhen Song, Huijun Huang, Liwei Fang, Hongli Zhang, Lijuan Pan, Naibo Hu, Shiqiang Qu, Yue Zhang, Gang Huang, Zhijian Xiao Tags: Correspondence Source Type: research

The exomic landscape of t(14;18) ‐negative diffuse follicular lymphoma with 1p36 deletion
Summary Predominantly diffuse t(14;18) negative follicular lymphoma (FL) with 1p36 deletion shows distinctive clinical, morphological and molecular features that distinguish it from classical FL. In order to investigate whether it possesses a unique mutation profile, we performed whole exome sequencing of six well‐characterised cases. Our analysis showed that the mutational landscape of this subtype is largely distinct from classical FL. It appears to harbour several recurrent mutations, affecting STAT6, CREBBP and basal membrane protein genes with high frequency. Our data support the view that this FL subtype should be ...
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Alberto Zam ò, Jordan Pischimarov, Heike Horn, German Ott, Andreas Rosenwald, Ellen Leich Tags: Short Report Source Type: research

Recent progress in understanding and manipulating haemoglobin switching for the haemoglobinopathies
Summary The major β‐haemoglobinopathies, sickle cell disease and β‐thalassaemia, represent the most common monogenic disorders worldwide and a steadily increasing global disease burden. Allogeneic haematopoietic stem cell transplantation, the only curative therapy, is only applied to a small minority of patients. Common clinical management strategies act mainly downstream of the root causes of disease. The observation that elevated fetal haemoglobin expression ameliorates these disorders has motivated longstanding investigations into the mechanisms of haemoglobin switching. Landmark studies over the last deca...
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Divya S. Vinjamur, Daniel E. Bauer, Stuart H. Orkin Tags: Review Source Type: research

Outcome of relapse after allogeneic HSCT in children with ALL enrolled in the ALL ‐SCT 2003/2007 trial
Summary Relapse remains the major cause of treatment failure in children with high‐risk acute lymphoblastic leukaemia (ALL) undergoing allogeneic haematopoietic stem‐cell transplantation (allo‐SCT). Prognosis is considered dismal but data on risk factors and outcome are lacking from prospective studies. We analysed 242 children with recurrence of ALL after first allo‐SCT enrolled in the Berlin‐Frankfurt‐Munster (BFM) ALL‐SCT‐BFM 2003 and ALL‐SCT‐BFM international 2007 studies. Median time from allo‐SCT to relapse was 7·7 months; median follow‐up from relapse after allo‐SCT until last f...
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Michaela Kuhlen, Andre M. Willasch, Jean ‐Hugues Dalle, Jacek Wachowiak, Isaac Yaniv, Marianne Ifversen, Petr Sedlacek, Tayfun Guengoer, Peter Lang, Peter Bader, Sabina Sufliarska, Adriana Balduzzi, Brigitte Strahm, Irene von Luettichau, Jessica I. Hoel Tags: Research Paper Source Type: research

From remission to cure: bypass or detour?
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Friedrich St ölzel Tags: Editorial Comment Source Type: research

A single ‐institution retrospective cohort study of first‐line R‐EPOCH chemoimmunotherapy for Richter syndrome demonstrating complex chronic lymphocytic leukaemia karyotype as an adverse prognostic factor
Summary Richter Syndrome, an aggressive lymphoma occurring in patients with chronic lymphocytic leukaemia (CLL), has a generally poor prognosis and anthracycline‐based chemoimmunotherapy regimens designed to treat de novo diffuse large B‐cell lymphoma achieve modest clinical benefit. R‐EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin) has demonstrated greater activity against aggressive B‐cell histologies but has not been studied in Richter Syndrome. We conducted a retrospective cohort study of 46 Richter Syndrome patients treated with first‐line R‐EPOCH at our institution bet...
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Kerry A. Rogers, Ying Huang, Amy S. Ruppert, Galena Salem, Deborah M. Stephens, Nyla A. Heerema, Leslie A. Andritsos, Farrukh T. Awan, John C. Byrd, Joseph M. Flynn, Kami J. Maddocks, Jeffrey A. Jones Tags: Research Paper Source Type: research

Prospective subgroup analyses of the randomized MCL ‐002 (SPRINT) study: lenalidomide versus investigator's choice in relapsed or refractory mantle cell lymphoma
Summary In the mantle cell lymphoma (MCL)‐002 study, lenalidomide demonstrated significantly improved median progression‐free survival (PFS) compared with investigator's choice (IC) in patients with relapsed/refractory MCL. Here we present the long‐term follow‐up data and results of preplanned subgroup exploratory analyses from MCL‐002 to evaluate the potential impact of demographic factors, baseline clinical characteristics and prior therapies on PFS. In MCL‐002, patients with relapsed/refractory MCL were randomized 2:1 to receive lenalidomide (25 mg/day orally on days 1–21; 28‐day cycles) or sing...
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Luca Arcaini, Thierry Lamy, Jan Walewski, David Belada, Jiri Mayer, John Radford, Wojciech Jurczak, Franck Morschhauser, Julia Alexeeva, Simon Rule, Jos é Cabeçadas, Elias Campo, Stefano A. Pileri, Tsvetan Biyukov, Meera Patturajan, Marie‐Laure Casade Tags: Research Paper Source Type: research

Obesity negatively impacts outcome in elderly female patients with aggressive B ‐cell lymphomas treated with R‐CHOP: results from prospective trials of the German high grade non‐Hodgkin's lymphoma trial group
Summary To study if obesity is a risk factor in elderly patients (>60 years) with aggressive B‐cell lymphoma, the outcomes of 576 elderly patients treated with rituximab in the RICOVER‐60 trial were analysed in a retrospective study with regard to body mass index (BMI) and gender. Of the 576 patients, 1% had low body weight (BMI 
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: K. Hohloch, B. Altmann, M. Pfreundschuh, M. Loeffler, N. Schmitz, F. Zettl, M. Ziepert, L. Tr ümper Tags: Research Paper Source Type: research

Bendamustine, bortezomib and rituximab produces durable complete remissions in patients with previously untreated, low grade lymphoma
Abstract This Phase II trial evaluated the efficacy of bendamustine, bortezomib and rituximab in patients with previously untreated low‐grade lymphoma. Eligible patients had low grade lymphoma with no previous systemic disease treatment. Treatment for all patients was given in 28‐day cycles for a maximum of 6 cycles. Patients received rituximab 375 mg/m2 intravenously (IV) on days 1, 8 and 15 of cycle 1 and day 1 of cycles 2–6; bendamustine 90 mg/m2 IV on days 1 and 2; and bortezomib 1·6 mg/m2 IV on days 1, 8 and 15. Patients were permitted to begin maintenance treatment with rituximab 6&nbs...
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Ian W. Flinn, Dana S. Thompson, Ralph V. Boccia, Gerald Miletello, Andrew Lipman, Douglas Flora, Daniel Cuevas, Steven W. Papish, Jesus G. Berdeja Tags: Research Paper Source Type: research

Aggressive lymphomas of the elderly: the DEVEC metronomic chemotherapy schedule fits the unfit
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 28, 2017 Category: Hematology Authors: Maria Christina Cox, Gerardo Musuraca, Roberta Battistini, Ivana Casaroli, Valerio Zoli, Paola Anticoli ‐Borza, Annalisa Arcari, Virginia Naso, Francesca di Landro, Francesca Fabbri, Agostino Tafuri, Guido Bocci, Francesco Merli Tags: Correspondence Source Type: research

Issue Information
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 27, 2017 Category: Hematology Tags: Issue Information Source Type: research

MYD88 wild ‐type Waldenstrom Macroglobulinaemia: differential diagnosis, risk of histological transformation, and overall survival
Summary MYD88 mutations are present in 95% of Waldenstrom Macroglobulinaemia (WM) patients, and support diagnostic discrimination from other IgM‐secreting B‐cell malignancies. Diagnostic discrimination can be difficult among suspected wild‐type MYD88 (MYD88WT) WM cases. We systematically reviewed the clinical, pathological and laboratory studies for 64 suspected MYD88WT WM patients. World Health Organization and WM consensus guidelines were used to establish clinicopathological diagnosis. Up to 30% of suspected MYD88WT WM cases had an alternative clinicopathological diagnosis, including IgM multiple myeloma. The esti...
Source: British Journal of Haematology - November 27, 2017 Category: Hematology Authors: Steven P. Treon, Joshua Gustine, Lian Xu, Robert J. Manning, Nicholas Tsakmaklis, Maria Demos, Kirsten Meid, Maria L. Guerrera, Manit Munshi, Gloria Chan, Jiaji Chen, Amanda Kofides, Christopher J. Patterson, Guang Yang, Xia Liu, Patricia Severns, Toni Du Tags: Research Paper Source Type: research

Platelets derived from fresh and cold ‐stored whole blood participate in clot formation in rats with acute traumatic coagulopathy
This study aimed to determine whether platelets derived from FWB and stored whole blood (SWB) contribute to clot formation in tissue injury after transfusion into coagulopathic rats with polytrauma/haemorrhage (T/H). The rats were resuscitated 1 h after trauma with FWB or SWB collected from green fluorescence protein (GFP) transgenic rats. After transfusion, a liver incision was made and the tissue was collected 10 min after injury to identify GFP+ platelets by immunohistochemistry. In comparison to FWB, platelet aggregation to adenosine diphosphate and protease‐activated receptor‐4 was reduced by 35% and 20%...
Source: British Journal of Haematology - November 22, 2017 Category: Hematology Authors: Xiaowu Wu, Daniel N. Darlington, Robbie K. Montgomery, Bin Liu, Jeffrey D. Keesee, Michael R. Scherer, Avi Benov, Jacob Chen, Andrew P. Cap Tags: research paper Source Type: research

The absolute percent deviation of IGHV mutation rather than a 98% cut ‐off predicts survival of chronic lymphocytic leukaemia patients treated with fludarabine, cyclophosphamide and rituximab
Summary The degree of somatic hypermutation, determined as percent deviation of immunoglobulin heavy chain gene variable region sequence from the germline (IGHV%), is an important prognostic factor in chronic lymphocytic leukaemia (CLL). Currently, a cut‐off of 2% deviation or 98% sequence identity to germline in IGHV sequence is routinely used to dichotomize CLL patients into mutated and unmutated groups. Because dissimilar IGHV% cut‐offs of 1–5% were identified in different studies, we wondered whether no cut‐off should be applied and IGHV% treated as a continuous variable. We analysed the significance of IGH...
Source: British Journal of Haematology - November 21, 2017 Category: Hematology Authors: Preetesh Jain, Graciela M. Nogueras Gonz ález, Rashmi Kanagal‐Shamanna, Uri Rozovski, Nawid Sarwari, Constantine Tam, William G. Wierda, Philip A. Thompson, Nitin Jain, Rajyalakshmi Luthra, Andres Quesada, Gabriela Sanchez‐Petitto, Alessandra Ferrajo Tags: Research Paper Source Type: research

Methotrexate ‐associated lymphoproliferative disorder masquerading as multiple cerebral metastases
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 21, 2017 Category: Hematology Authors: Ikuo Matsuda, Seiichi Hirota Tags: Images in Haematology Source Type: research

Issue Information
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 19, 2017 Category: Hematology Tags: Issue Information Source Type: research

Inter ‐individual variability in phospholipid‐dependent interference of C‐reactive protein on activated partial thromboplastin time
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 16, 2017 Category: Hematology Authors: Lale Erdem ‐Eraslan, Jacques J. H. Hens, André P. van Rossum, Marieke A. M. Frasa, Jeffrey F. W. Keuren Tags: Correspondence Source Type: research

Is food intolerance a factor underlying chronic immune thrombocytopenia (ITP)?
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 16, 2017 Category: Hematology Authors: Claire A. Batty, John O. Hunter, Jenny Woolner, Trevor Baglin, Claire Turner Tags: Correspondence Source Type: research

Interleukin ‐12 and its procoagulant effect on erythrocytes, platelets and fibrin(ogen): the lesser known side of inflammation
Summary Inflammation, with its associated inflammatory molecules, is integral to most chronic diseases, including the various cardiovascular diseases. Interleukin 12 (IL12) is one of the inflammatory cytokines that is upregulated during inflammation; however, we know very little about its exact effect on red blood cells (RBCs), platelets and fibrin(ogen). IL12 is an important pleiotropic cytokine in early inflammatory responses and has potent immunomodulatory, antitumour and anti‐infection activity. Here we investigate how low levels of circulating IL12, comparable to levels found during chronic inflammation, affect coag...
Source: British Journal of Haematology - November 16, 2017 Category: Hematology Authors: Martin J. Page, Janette Bester, Etheresia Pretorius Tags: Research Paper Source Type: research

Clinical features and outcomes of secondary intraocular lymphoma
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 16, 2017 Category: Hematology Authors: Ayako Karakawa, Kazuki Taoka, Toshikatsu Kaburaki, Rie Tanaka, Aya Shinozaki ‐Ushiku, Hiroyuki Hayashi, Akiko Miyagi‐Maeshima, Yu Nishimura, Toshimasa Uekusa, Yui Kojima, Masashi Fukayama, Mineo Kurokawa, Makoto Aihara Tags: Correspondence Source Type: research

The full spectrum of Castleman disease: 273 patients studied over 20  years
Summary The spectrum of Castleman disease (CD) has considerably extended since its first description in 1956. Recently, an international collaborative working group has reached consensus on the diagnostic criteria and classification of CD. We herein report 273 patients with lymph node histopathology consistent with CD and investigate the newly established diagnostic criteria. Twenty of these patients with Castleman‐like histopathology were removed from analyses, because they were diagnosed with an exclusionary disorder (18 with haematological malignancy). Among the 253 remaining patients, 57 were considered unicentric CD...
Source: British Journal of Haematology - November 16, 2017 Category: Hematology Authors: Eric Oksenhendler, David Boutboul, David Fajgenbaum, Adrien Mirouse, Claire Fieschi, Marion Malphettes, Laetitia Vercellino, V éronique Meignin, Laurence Gérard, Lionel Galicier Tags: Research Paper Source Type: research

Castleman disease: delineating the spectrum
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 16, 2017 Category: Hematology Authors: Musa Alzahrani, Heather A. Leitch Tags: Editorial comment Source Type: research

Potentially inappropriate medication use in elderly non ‐Hodgkin lymphoma patients is associated with reduced survival and increased toxicities
We report here for the first time that potentially inappropriate medication use was associated with reduced progression‐free survival and overall survival, and increased ≥ grade 3 treatment‐related toxicities in multivariate analysis. (Source: British Journal of Haematology)
Source: British Journal of Haematology - November 16, 2017 Category: Hematology Authors: Richard J. Lin, Helen Ma, Robin Guo, Andrea B. Troxel, Catherine S. Diefenbach Tags: Short Report Source Type: research

Bone marrow Dikkopf ‐1 levels are a new independent risk factor for progression in patients with smouldering myeloma
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 16, 2017 Category: Hematology Authors: Benedetta Dalla Palma, Valentina Marchica, Mario Pedrazzoni, Fabrizio Accardi, Laura Notarfranchi, Matteo Goldoni, Federica De Luca, Federica Costa, Paola Storti, Denise Toscani, Gabriella Sammarelli, Sabrina Bonomini, Franco Aversa, Nicola Giuliani Tags: Correspondence Source Type: research

Enumeration and characterization of circulating multiple myeloma cells in patients with plasma cell disorders
Summary We have developed an automated assay to enumerate and characterize circulating multiple myeloma cells (CMMC) from peripheral blood of patients with plasma cell disorders. CMMC show expression of genes characteristic of myeloma and fluorescence in situ hybridisation results on CMMC correlated well with bone marrow results. We enumerated CMMC from over 1000 patient samples including separate cohorts of newly diagnosed multiple myeloma and high/intermediate risk smouldering multiple myeloma (SMM) with clinical follow‐up data. In newly diagnosed myeloma patient samples, CMMC counts correlated with other clinical...
Source: British Journal of Haematology - November 5, 2017 Category: Hematology Authors: Brad Foulk, Mike Schaffer, Steve Gross, Chandra Rao, Denis Smirnov, Mark C. Connelly, Shalini Chaturvedi, Manjula Reddy, Greg Brittingham, Marielena Mata, Madeline Repollet, Claudia Rojas, Daniel Auclair, Mary DeRome, , Brendan Weiss, Amy K. Sasser Tags: Research Paper Source Type: research

Transplant results in adults with Fanconi anaemia
Summary The outcomes of adult patients transplanted for Fanconi anaemia (FA) have not been well described. We retrospectively analysed 199 adult patients with FA transplanted between 1991 and 2014. Patients were a median of 16 years of age when diagnosed with FA, and underwent transplantation at a median age of 23 years. Time between diagnosis and transplant was shortest (median 2 years) in those patients who had a human leucocyte antigen identical sibling donor. Fifty four percent of patients had bone marrow (BM) failure at transplantation and 46% had clonal disease (34% myelodysplasia, 12% acute leukaemia)...
Source: British Journal of Haematology - November 2, 2017 Category: Hematology Authors: Marc Bierings, Carmem Bonfim, Regis Peffault De Latour, Mahmoud Aljurf, Parinda A. Mehta, Cora Knol, Farid Boulad, Abdelghani Tbakhi, Albert Esquirol, Grant McQuaker, Gulsan A. Sucak, Tarek B. Othman, Constantijn J. M. Halkes, Ben Carpenter, Dietger Niede Tags: Research Paper Source Type: research

Pathogenesis and therapeutic targeting of aberrant MYC expression in haematological cancers
Summary Identifying and therapeutically targeting cancer cell liabilities is of utmost importance in order to improve the treatment of patients with malignancies of poor prognosis. The MYC family genes (MYC, MYCN and MYCL) are among the most deregulated proto‐oncogenes in human cancer. Aberrant MYC expression is frequently associated with poor prognosis. Although many aspects of MYC‐mediated tumour biology are well characterized, there are currently no effective means for targeting MYC in a specific manner that have been established for clinical use. This review first discusses the role of MYC in the pathogenesis of ha...
Source: British Journal of Haematology - November 1, 2017 Category: Hematology Authors: Markus Schick, Stefan Habringer, Jonas A. Nilsson, Ulrich Keller Tags: Review Source Type: research

Screening for ASXL1 and SRSF2 mutations is imperative for treatment decision ‐making in otherwise low or intermediate‐1 risk patients with myelofibrosis
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 1, 2017 Category: Hematology Authors: Ayalew Tefferi, Terra L. Lasho, Curtis A. Hanson, Rhett P. Ketterling, Naseema Gangat, Animesh Pardanani Tags: Correspondence Source Type: research

The value of cold storage whole blood platelets in trauma resuscitation is like real estate: a function of ‘location, location, location’
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 1, 2017 Category: Hematology Authors: Mark Walsh, Jacob Shreve, Scott Thomas Tags: Editorial Comment Source Type: research

Safety and efficacy of pomalidomide, dexamethasone and pegylated liposomal doxorubicin for patients with relapsed or refractory multiple myeloma
Summary Immunomodulatory drugs including thalidomide, lenalidomide (LEN) and pomalidomide (POM), are effective for treating multiple myeloma (MM). POM has shown enhanced efficacy with dexamethasone (DEX). Pegylated liposomal doxorubicin (PLD) with bortezomib is US Food and Drug Administration‐approved for treating MM. PLD with LEN or thalidomide has shown efficacy for MM patients. LEN with DEX, PLD and bortezomib achieves high response rates. We evaluated the combination of POM with DEX 40 mg and PLD 5 mg/m2 with the latter two drugs administered on days 1, 4, 8 and 11 on a 28‐day cycle for the treatment of r...
Source: British Journal of Haematology - November 1, 2017 Category: Hematology Authors: Alexa Cohen, Tanya M. Spektor, Laura Stampleman, Alberto Bessudo, Peter J. Rosen, Leonard M. Klein, Thomas Woliver, Marshall Flam, Shahrooz Eshaghian, Youram Nassir, Tina Maluso, Regina A. Swift, Robert Vescio, James R. Berenson Tags: Research Paper Source Type: research

Erythrophagocytosis by T ‐cell lymphoma cells in a patient with hereditary spherocytosis post‐splenectomy
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 1, 2017 Category: Hematology Authors: Ithiel J. Frame, Jared Coberly, Franklin S. Fuda, Mingyi Chen Tags: Images in Haematology Source Type: research

Fetal haemoglobin induction in sickle cell disease
Summary Fetal haemoglobin (HbF, α2γ2) induction has long been an area of investigation, as it is known to ameliorate the clinical complications of sickle cell disease (SCD). Progress in identifying novel HbF‐inducing strategies has been stymied by limited understanding of gamma (γ)–globin regulation. Genome‐wide association studies (GWAS) have identified variants in BCL11A and HBS1L‐MYB that are associated with HbF levels. Functional studies have established the roles of BCL11A, MYB, and KLF1 in γ–globin regulation, but this information has not yielded new pharmacological agents. S...
Source: British Journal of Haematology - November 1, 2017 Category: Hematology Authors: Alireza Paikari, Vivien A. Sheehan Tags: Review Source Type: research

Pyridoxine ‐sensitive X‐linked ‘sideroblastic’ anaemia in the absence of ring sideroblasts – molecular diagnosis
(Source: British Journal of Haematology)
Source: British Journal of Haematology - November 1, 2017 Category: Hematology Authors: Thomas Creasey, Tina Biss, John Lambert, Frances Smith, Barnaby Clark, Peter Carey Tags: Images in Haematology Source Type: research

Quantitative mass spectrometry of human reticulocytes reveal proteome ‐wide modifications during maturation
Summary Erythropoiesis is marked by progressive changes in morphological, biochemical and mechanical properties of erythroid precursors to generate red blood cells (RBC). The earliest enucleated forms derived in this process, known as reticulocytes, are multi‐lobular and spherical. As reticulocytes mature, they undergo a series of dynamic cytoskeletal re‐arrangements and the expulsion of residual organelles, resulting in highly deformable biconcave RBCs (normocytes). To understand the significant, yet neglected proteome‐wide changes associated with reticulocyte maturation, we undertook a quantitative proteomics appro...
Source: British Journal of Haematology - November 1, 2017 Category: Hematology Authors: Trang T. T. Chu, Ameya Sinha, Benoit Malleret, Rossarin Suwanarusk, Jung E. Park, Renugah Naidu, Rupambika Das, Bamaprasad Dutta, Seow Theng Ong, Navin K. Verma, Jerry K. Chan, Fran çois Nosten, Laurent Rénia, Siu K. Sze, Bruce Russell, Rajesh Chandramo Tags: Research Paper Source Type: research

Role of 99mTc ‐DPD scintigraphy in imaging extra‐cardiac light chain (AL) amyloidosis
(Source: British Journal of Haematology)
Source: British Journal of Haematology - October 30, 2017 Category: Hematology Authors: Sajitha Sachchithanantham, David F. Hutt, Ann ‐Marie Quigley, Philip Hawkins, Ashutosh D. Wechalekar Tags: Correspondence Source Type: research

Optimizing the management of hereditary haemochromatosis: the value of MRI R2* quantification to predict and monitor body iron stores
(Source: British Journal of Haematology)
Source: British Journal of Haematology - October 30, 2017 Category: Hematology Authors: Manuela Fran ça, Luis Martí‐Bonmatí, Sara Silva, Carla Oliveira, Ángel Alberich Bayarri, Filipa Vilas Boas, Helena Pessegueiro‐Miranda, Graça Porto Tags: Correspondence Source Type: research

A new heterozygous mutation in GP1BA gene responsible for macrothrombocytopenia
(Source: British Journal of Haematology)
Source: British Journal of Haematology - October 30, 2017 Category: Hematology Authors: Dorsaf Ghalloussi, No émie Saut, Denis Bernot, Xavier Pillois, Philippe Rameau, Gérard Sébahoun, Marie‐Christine Alessi, Hana Raslova, Véronique Baccini Tags: Correspondence Source Type: research

Efficacy of biological agents in the treatment of Erdheim ‐Chester disease
(Source: British Journal of Haematology)
Source: British Journal of Haematology - October 30, 2017 Category: Hematology Authors: Gaurav Goyal, Mithun V. Shah, Timothy G. Call, Mark R. Litzow, Alexandra P. Wolanskyj ‐Spinner, Matthew J. Koster, W. Oliver Tobin, Robert Vassallo, Jay H. Ryu, Christopher C. Hook, William J. Hogan, Ronald S. Go Tags: Correspondence Source Type: research

What is currently known about the genetics of venous thromboembolism at the dawn of next generation sequencing technologies
Summary Venous thromboembolism (VTE) has a strong genetic component. This review summarizes what is known at the seventeen genes that are now well established to harbour VTE‐associated genetic variants. In addition, it discusses additional candidate genes that deserve further validation before being claimed as VTE associated genes. Finally, several research strategies are briefly described to identify other molecular determinants of the disease. (Source: British Journal of Haematology)
Source: British Journal of Haematology - October 29, 2017 Category: Hematology Authors: David ‐Alexandre Trégouët, Pierre‐Emmanuel Morange Tags: Review Source Type: research