12,213 results match your criteria Journal of Hematology & Oncology [Journal]


Rapid growth is a dominant predictor of hepcidin suppression and declining ferritin in Gambian infants.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

MRC Unit The Gambia at the London School of Hygiene and Tropical Medicine, Banjul, The Gambia.

Iron deficiency and iron deficiency anemia are highly prevalent in low-income countries, especially among young children. Hepcidin is the major regulator of systemic iron homeostasis. It controls dietary iron absorption, dictates whether absorbed iron is made available in circulation for erythropoiesis and other iron-demanding processes, and predicts response to oral iron supplementation. Read More

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http://dx.doi.org/10.3324/haematol.2018.210146DOI Listing
February 2019
1 Read
5.868 Impact Factor

The novel CD19-targeting antibody-drug conjugate huB4-DGN462 shows improved anti-tumor activity than SAR3419 in CD19-positive lymphoma and leukemia models.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Università della Svizzera italiana, Institute of Oncology Research;

Antibody-drug conjugates (ADCs) are a novel way to deliver potent cytotoxic compounds to cells expressing a specific antigen. Four ADCs targeting CD19, including SAR3419 (coltuximab ravtansine), have entered clinical development. Here, we present huB4-DGN462, a novel ADC based on the SAR3419 anti-CD19 antibody linked via sulfo-SPDB to the potent DNA-alkylating agent DGN462. Read More

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http://dx.doi.org/10.3324/haematol.2018.211011DOI Listing
February 2019

Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma genetically unrelated to Burkitt lymphoma.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Institut Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain;

Burkitt-like lymphoma with 11q aberration is characterized by pathological features and gene expression profile resembling Burkitt lymphoma but lack MYC rearrangement and carries an 11q-arm aberration with proximal gains and telomeric losses. Whether these lymphomas are a distinct category or a particular variant of other recognized entities is controversial. To improve the understanding of Burkitt-like lymphoma with 11q aberration we have performed an analysis of copy number alterations and targeted sequencing of a large panel of B-cell lymphoma related genes in 11 cases. Read More

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http://dx.doi.org/10.3324/haematol.2018.207928DOI Listing
February 2019
1 Read

A phase II study of guadecitabine in higher-risk myelodysplastic syndrome and low blast count acute myeloid leukemia after azacitidine failure.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Hématologie clinique, Hôpital Saint-Louis, Paris, France;

High-risk myelodysplastic syndrome/acute myeloid leukemia patients have a very poor survival after azacitidine failure. Guadecitabine (SGI-110) is a novel subcutaneous hypomethylating agent, which results in extended decitabine exposure. This multicenter phase II study evaluated the efficacy and safety of guadecitabine in high-risk myelodysplastic syndrome and low blast count acute myeloid leukemia patients refractory or relapsing after azacitidine. Read More

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http://dx.doi.org/10.3324/haematol.2018.207118DOI Listing
February 2019
1 Read

Once-weekly versus twice-weekly carfilzomib in patients with newly diagnosed multiple myeloma: a pooled analysis of two phase 1/2 studies.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Division of Hematology, University of Torino,AOU Città della Salute e della Scienza di Torino.

Twice-weekly carfilzomib is approved at 27 and 56 mg/m to treat relapsed multiple myeloma patients. In the phase 3 study A.R. Read More

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http://dx.doi.org/10.3324/haematol.2018.208272DOI Listing
February 2019
1 Read

Long-term outcome after allogeneic hematopoietic cell transplantation for myelofibrosis.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

University Hospital Eppendorf, Hamburg, Germany.

Allogeneic hematopoietic stem cell transplant remains the only curative treatment for myelofibrosis. Most post-transplantation events occur during the first 2 years and hence we aimed to analyze the outcome of 2-year disease-free survivors. 1055 patients with myelofibrosis transplanted between 1995 and 2014 and registered in the registry of the European Society for Blood and Marrow Transplantation were included. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.205211DOI Listing
February 2019
2 Reads

Clonal evolution in myeloma: the impact of maintenance lenalidomide and depth of response on the genetics and sub-clonal structure of relapsed disease in uniformly treated newly diagnosed patients.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

University of Arkansas for Medical Sciences, Myeloma Institute;

The emergence of treatment resistant sub-clones is a key feature of relapse in multiple myeloma. Therapeutic attempts to extend remission and prevent relapse include the maximisation of response and use of maintenance therapy. We used whole exome sequencing to study the genetics of paired presentation and relapse samples from 56 newly diagnosed patients, following induction therapy, randomised to receive either lenalidomide maintenance or observation as part of the Myeloma XI trial. Read More

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http://dx.doi.org/10.3324/haematol.2018.202200DOI Listing
February 2019

Anti-apoptotic BCL2L2 increases megakaryocyte proplatelet formation in cultures of human cord blood.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Division of Hematology and Program in Molecular Medicine, University of Utah, Salt Lake City, USA

Apoptosis is a recognized limitation for generating large numbers of megakaryocytes in culture. The responsible apoptosis genes have been rigorously studied in vivo in mice, but are poorly characterized in human culture systems. As CD34+ cells isolated from human umbilical vein cord blood were differentiated into megakaryocytes in culture, two distinct cell populations were identified by flow cytometric forward and side scatter: larger size, lower granularity and smaller size, higher granularity. Read More

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http://dx.doi.org/10.3324/haematol.2018.204685DOI Listing
February 2019

Reactivation of hepatitis B infection in patients with haematological disorders.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Institute of Liver Studies, King's College Hospital, London, UK.

Hepatitis B reactivation is the reappearance or rise of hepatitis B virus (HBV) DNA in patients with past or chronic HBV infection, usually occurring in the context of immunosuppression. HBV reactivation has been most commonly reported in patients with haematological disorders, with potentially serious and life-threatening consequences. In this review, we discuss the basis and presentation of HBV reactivation, risk factors in terms of the host, the virus and the immunosuppression regimen, focusing on specific treatments relevant to haematology patients. Read More

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http://dx.doi.org/10.3324/haematol.2018.210252DOI Listing
February 2019
1 Read

Platelet glycoprotein VI and C-type lectin-like receptor 2 deficiency accelerates wound healing by impairing vascular integrity in mice.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

University of Birmingham

Platelets promote wound healing by forming a vascular plug and by secreting growth factors and cytokines. Glycoprotein (GP)VI and C-type lectin-like receptor (CLEC)-2 signal through a (hem)-immunoreceptor tyrosine-based activation motif, which induce platelet activation. GPVI and CLEC-2 support vascular integrity during inflammation in the skin through regulation of leukocyte migration and function, and by sealing sites of vascular damage. Read More

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http://dx.doi.org/10.3324/haematol.2018.208363DOI Listing
February 2019
1 Read

Fecal microbiota transplantation before or after allogeneic hematopoietic transplantation in patients with hematological malignancies carrying multidrug-resistance bacteria.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Department of Hematology, Saint Antoine Hospital, Paris, France.

Fecal microbiota transplantation is an effective treatment in recurrent Clostridium difficile infection. Promising results to eradicate multidrug-resistant bacteria have also been reported with this procedure, but there are safety concerns in immunocompromised patients. We report results in 10 adult patients colonized with multidrug-resistant bacteria, undergoing fecal microbiota transplantation before (n=4) or after (n=6) allogeneic hematopoietic stem cell transplantation for hematologic malignancies. Read More

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http://dx.doi.org/10.3324/haematol.2018.198549DOI Listing
February 2019
3 Reads

Sequential therapy for patients with primary refractory acute myeloid leukemia - historical prospective analysis of the German and Israeli experience.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Department I of Internal Medicine, University of Cologne, Germany.

Primary refractory acute myeloid leukemia is associated with a dismal prognosis. The fludarabine, amsacrine, and cytarabine-reduced-intensity conditioning protocol (total body irradiation or treosulfan-based) has been described to be an effective approach in patients with refractory leukemia undergoing allogeneic hematopoietic cell transplantation . A modified protocol (without amsacrine) has been also used recently. Read More

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http://dx.doi.org/10.3324/haematol.2018.203869DOI Listing
February 2019

Ectopic ATP synthase β subunit proteins on human leukemia cell surface interact with platelets by binding glycoprotein IIb.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Lab of Thrombosis and Hemostasis, The First Affiliated Hospital of Soochow University, Suzhou, China;

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http://dx.doi.org/10.3324/haematol.2019.216390DOI Listing
February 2019

Incidence of sleep problems and their mediating role on depression and anxious preoccupation in patients with resected, non-advanced cancer: data from NEOcoping study.

Clin Transl Oncol 2019 Feb 2. Epub 2019 Feb 2.

Department of Medical Oncology, Hospital Universitario Central of Asturias, Avenida Roma, sn, 33011, Oviedo, Spain.

Background: Our study analyzes the incidence of sleep problems and their mediating role on depression and anxious preoccupation in patients with resected, non-advanced cancer.

Methods: A multi-institutional, prospective, observational study was conducted with 750 participants of 14 hospitals in Spain. Participants' socio-demographic and clinical characteristics were collected using a standardized self-report form and using EORTC QoL-QLQ-C30, BSI, Mini-MAC questionnaires. Read More

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http://dx.doi.org/10.1007/s12094-018-02018-2DOI Listing
February 2019
1 Read

Prevention of serious infections in hereditary hemorrhagic telangiectasia: roles for prophylactic antibiotics, the pulmonary capillaries-but not vaccination.

Haematologica 2019 Feb;104(2):e85-e86

Hospices Civils de Lyon, Hôpital Femme-Mère-Enfants, Service de Génétique, and VASCERN HHT European Reference Centre/centre de Référence pour la maladie de Rendu-Osler, France, and Université de Lyon, Faculté de médecine, France.

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http://dx.doi.org/10.3324/haematol.2018.209791DOI Listing
February 2019
1 Read

The stepchild in myeloma treatments: is allogeneic transplantation not so bad after all?

Haematologica 2019 Feb;104(2):222-225

Department of Oncology/Hematology, A.O.U. Città della Salute e della Scienza di Torino, Presidio Molinette, Torino, Italy

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http://dx.doi.org/10.3324/haematol.2018.206987DOI Listing
February 2019

Another step forward in the 20-year history of mutations in chronic lymphocytic leukemia.

Haematologica 2019 Feb;104(2):219-221

Hematology, Department of Translational and Precision Medicine, 'Sapienza' University, Rome, Italy

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.207399DOI Listing
February 2019
2 Reads

Milk and the "Grandmother Effect"- a new contribution to the legacy of Ray Owen.

Haematologica 2019 Feb;104(2):216-218

University of Wisconsin, Madison, WI, USA

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http://dx.doi.org/10.3324/haematol.2018.207340DOI Listing
February 2019

Antithymocyte globulin and cyclosporin: standard of care also for older patients with aplastic anemia.

Haematologica 2019 Feb;104(2):215-216

Fondazione Policlinico Universitario A. Gemelli IRCCS, Universita' Cattolica del Sacro Cuore, Rome, Italy

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http://dx.doi.org/10.3324/haematol.2018.207167DOI Listing
February 2019

Tracking of myeloid malignancies by targeted analysis of successive DNA methylation at neighboring CG dinucleotides.

Haematologica 2019 Jan 31. Epub 2019 Jan 31.

Helmholtz-Institute for Biomedical Engineering, RWTH Aachen University, Aachen, Germany;

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http://dx.doi.org/10.3324/haematol.2018.209734DOI Listing
January 2019
1 Read

IgG4-related disease: what a hematologist needs to know.

Haematologica 2019 Jan 31. Epub 2019 Jan 31.

Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, Canada.

IgG4-related disease is a fibroinflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. This review focuses on the hematological manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia. Read More

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http://dx.doi.org/10.3324/haematol.2018.205526DOI Listing
January 2019
2 Reads

Asparagine levels in the cerebrospinal fluid of children with acute lymphoblastic leukemia treated with PEG-Asparaginase in the induction phase of the AIEOP-BFM ALL 2009 study.

Haematologica 2019 Jan 31. Epub 2019 Jan 31.

University Childrens Hospital of Münster, Münster, Germany.

Asparagine levels in cerebrospinal fluid and serum asparaginase activities were monitored in children with acute lymphoblastic leukemia treated with PEG-asparaginase. The drug was given intravenously at 2,500 IU/m2 on days 12 and 26. Serum and cerebrospinal fluid samples obtained on days 33 and 45 were centrally analyzed. Read More

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http://dx.doi.org/10.3324/haematol.2018.206433DOI Listing
January 2019

Recognition of hemophagocytic lymphohistiocytosis in sickle cell vasoocclusive crises is a potentially lifesaving diagnosis.

Haematologica 2019 Jan 31. Epub 2019 Jan 31.

Department of Medicine, Boston University School of Medicine, Boston MA, USA;

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http://dx.doi.org/10.3324/haematol.2018.206458DOI Listing
January 2019
1 Read

Blockade of crizotinib-induced BCL2 elevation in ALK-positive anaplastic large cell lymphoma triggers autophagy associated with cell death.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

CRCT, INSERM U1037

Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphomas are tumors that carry translocations involving the ALK gene at the 2p23 locus, leading to the expression of ALK tyrosine kinase fusion oncoproteins. Amongst hematological malignancies, these lymphomas are particular in that they express very low levels of B-Cell Lymphoma 2 (BCL2), a recognized inhibitor of apoptosis and autophagy, two processes that share complex interconnections. We have previously shown that treatment of ALK-positive anaplastic large cell lymphoma cells with the ALK tyrosine kinase inhibitor crizotinib induces autophagy as a pro-survival response. Read More

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http://dx.doi.org/10.3324/haematol.2017.181966DOI Listing
January 2019
4 Reads

Recurrent stroke: the role of thrombophilia in a large international pediatric stroke population.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Institute of Clinical Chemistry, University Hospital Kiel-Lübeck, Germany;

Risk factors for arterial ischaemic stroke in children include vasculopathy and prothrombotic risk factors but their relative importance to recurrent stroke is uncertain. Data on recurrent stroke from the databases held in Canada (Toronto), Germany (Kiel-Lubeck/Munster), and UK (London/Southampton) were pooled. Data were available from 894 patients aged 1 month to 18 years at first stroke (median age 6 years) with a median follow-up of 35 months. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.211433DOI Listing
January 2019
4 Reads

Characteristics and outcomes of patients with essential thrombocythemia or polycythemia vera diagnosed before 20 years of age, a systematic review.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Department of Hematology, Guy and St Thomas NHS Trust, London, United Kingdom.

Although it is well known that myeloproliferative neoplasms occur in younger patients, few large cohorts of such patients have been reported. Thus, our knowledge is limited especially for children and young adults about circumstances of diagnosis, outcome and treatment. We performed a systematic review of cases published since 2005, concerning patients aged below 20-years at the time of diagnosis of essential thrombocythemia or polycythemia vera. Read More

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http://dx.doi.org/10.3324/haematol.2018.200832DOI Listing
January 2019
1 Read

Enhanced hemato-endothelial specification during human embryonic differentiation through developmental cooperation between AF4-MLL and MLL-AF4 fusions.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Josep Carreras Leukemia Research Institute and Department of Biomedicine, School of Medicine;

The t(4;11)(q21;q23) translocation is associated with high-risk infant pro-B-cell acute lymphoblastic leukemia and arises prenatally during embryonic/fetal hematopoiesis. The developmental/pathogenic contribution of the t(4;11)-resulting MLL-AF4 (MA4) and AF4-MLL (A4M) fusions remains unclear; MA4 is always expressed in t(4;11)+ B-cell acute lymphoblastic leukemia patients, but the reciprocal fusion A4M is expressed in only half of the patients. Because prenatal leukemogenesis manifests as impaired early hematopoietic differentiation, we took advantage of well-established human embryonic stem cell-based hematopoietic differentiation models to study whether the A4M fusion cooperates with MA4 during early human hematopoietic development. Read More

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http://dx.doi.org/10.3324/haematol.2018.202044DOI Listing
January 2019
1 Read

All-trans retinoic acid protects mesenchymal stem cells from immune thrombocytopenia by regulating the complement-IL-1β loop.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Peking University People's Hospital, Peking University Institute of Hematology;

Peripheral enhanced complement activation has long been considered as one of the major pathogenesis of immune thrombocytopenia. Impaired bone marrow microenvironment, especially the dysfunction of mesenchymal stem cells, has been observed in patients with immune thrombocytopenia. However, the potential role of the complement system involved in impaired bone marrow microenvironment remains poorly understood. Read More

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http://dx.doi.org/10.3324/haematol.2018.204446DOI Listing
January 2019
1 Read
5.868 Impact Factor

Unravelling the cellular origin and clinical prognostic markers of infant B-cell acute lymphoblastic leukemia using genome-wide analysis.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Princess Maxima Center for Paediatric Oncology, Utrecht, The Netherlands.

B-cell acute lymphoblastic leukemia is the commonest childhood cancer. In infants, B-cell acute lymphoblastic leukemia remains fatal, especially in patients with t(4;11), present in ~80% of cases. The pathogenesis of t(4;11)/KMT2A-AFF1+ (MLL-AF4+) infant B-cell acute lymphoblastic leukemia remains difficult to model, and the pathogenic contribution in cancer of the reciprocal fusions resulting from derivative translocated-chromosomes remains obscure. Read More

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http://dx.doi.org/10.3324/haematol.2018.206375DOI Listing
January 2019

ZEB2 and LMO2 drive immature T-cell lymphoblastic leukemia via distinct oncogenic mechanisms.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Rady Faulty of Health Sciences, University of Manitoba, Winnipeg, Canada.

ZEB1 and ZEB2 are structurally related E-box binding homeobox transcription factors that induce epithelial to mesenchymal transitions during development and disease. As such, they regulate cancer cell invasion, dissemination and metastasis of solid tumors. In addition, their expression is associated with the gain of cancer stem cell properties and resistance to therapy. Read More

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http://dx.doi.org/10.3324/haematol.2018.207837DOI Listing
January 2019
1 Read

Bone marrow niches of germline FANCC/FANCG deficient mice enable efficient and durable engraftment of hematopoietic stem cells after transplantation.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Comprehensive Bone Marrow Failure Center, Children Hospital of Philadelphia. Philadelphia, PA

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http://dx.doi.org/10.3324/haematol.2018.202143DOI Listing
January 2019

A novel combinatorial technique for simultaneous quantification of oxygen radicals and aggregation reveals unexpected redox patterns in the activation of platelets by different physiopathological stimuli.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Institute of Biomedical & Clinical Science, University of Exeter Medical School, Exeter, UK;

The regulation of platelets by oxidants is critical for vascular health and may explain thrombotic complications in diseases such as diabetes and dementia, but remains poorly understood. Here, we describe a novel technique combining electron paramagnetic resonance spectroscopy and turbidimetry, which has been utilised to monitor simultaneously platelet activation and oxygen radical generation. This technique has been used to investigate the redox-dependence of human and mouse platelets. Read More

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http://dx.doi.org/10.3324/haematol.2018.208819DOI Listing
January 2019
1 Read

Clinical and biological features in PIEZO1-hereditary xerocytosis and Gardos-channelopathy: A retrospective series of 126 patients.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

EA 4666 HEMATIM UPJV and Service Hématologie Biologique, CHU Amiens, France

We describe clinical, hematological and genetic characteristics of a retrospective series of 126 subjects from 64 families with hereditary xerocytosis. Twelve patients from 6 families carried a KCNN4 mutation, 5 recurrent p.Arg352His mutations and one new deletion at the exon 7-intron 7 junction. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.205328DOI Listing
January 2019
4 Reads

Optimized EBMT transplant-specific risk score in myelodysplastic syndromes after allogeneic stem-cell transplantation.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

University Medical Center Hamburg-Eppendorf;

Here, we aimed to develop and validate a clinical and transplant-specific prognostic score in a large cohort of patients with myelodysplastic syndromes reported to the European Society for Blood and Marrow Transplantation registry. A Cox model was fitted to detect clinical and transplant-related variables prognostic of outcome. Then, cross-validation was assessed to evaluate the validity and consistency of the model. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.200808DOI Listing
January 2019
3 Reads

Epigenomic profiling of myelofibrosis reveals widespread DNA methylation changes in enhancer elements and ZFP36L1 as a potential tumor suppressor gene epigenetically regulated.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Centro de Investigacion Medica Aplicada, Universidad de Navarra, Pamplona, Spain;

In this study we have interrogated the DNA methylome of myelofibrosis patients using high-density DNA methylation arrays. We detected 35,215 differentially methylated CpGs corresponding to 10,253 genes between myelofibrosis patients and healthy controls. These changes were present both in primary and secondary myelofibrosis, which showed no differences between them. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.204917DOI Listing
January 2019
4 Reads

Deep sequencing of bone marrow microenvironments of patients with del(5q) myelodysplastic syndrome reveals imprints of antigenic selection as well as generation of novel T cell clusters as response pattern to lenalidomide.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Department of Haematology and Oncology, University Hospital Halle (Saale), Halle (Saale), Germany

In del(5q) myelodysplastic syndromes, lenalidomide is believed to reverse anergic T cell immunity in the bone marrow resulting in suppression of the del(5q)-clone. Here, we used next-generation sequencing of immunoglobulin heavy chain (IGH) and T cell receptor beta (TRB) rearrangements in bone marrow residing and peripheral blood circulating lymphocytes of patients with del(5q) myelodysplastic syndromes to assess the immune architecture and track adaptive immune responses during treatment with lenalidomide. The baseline bone marrow B cell space in patients was comparable to age-matched healthy donors in terms of gene usage and IGH clonality, but showed a higher percentage of hypermutated IGH sequences, indicating an extended number of antigen-experienced B lineage cells. Read More

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http://dx.doi.org/10.3324/haematol.2018.208223DOI Listing
January 2019
1 Read

Performance analysis of AL amyloidosis cardiac biomarker staging systems with special focus on renal failure and atrial arrhythmia.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Division of Hematology/Oncology, Heidelberg University Hospital, Germany;

Systemic light chain amyloidosis is a rare and life-threatening disorder, where accurate risk stratification is crucial. Current cardiac staging systems (MAYO2004, MAYO3b, MAYO2012) are mainly based on biomarkers, which have uncertain reliability in the context of atrial fibrillation, arrhythmia or pacemaker stimulation as well as renal insufficiency. We aimed to compare the performance of the established staging systems with particular regard to these comorbidities in 1224 patients with systemic light chain amyloidosis diagnosed at our center from July 2002 until March 2017. Read More

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http://dx.doi.org/10.3324/haematol.2018.205336DOI Listing
January 2019
2 Reads

Hypoxia attenuates inflammation-induced hepcidin production during experimental human endotoxaemia.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Radboud University Nijmegen Medical Center, Nijmegen, The Netherland

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http://dx.doi.org/10.3324/haematol.2018.202796DOI Listing
January 2019

Glucocorticoids and selumetinib are highly synergistic in RAS pathway mutated childhood acute lymphoblastic leukemia through upregulation of BIM.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Newcastle Cancer Centre, Newcastle University, Newcastle upon Tyne, UK;

New drugs are needed for relapsed acute lymphoblastic leukemia and preclinical evaluation of the MEK inhibitor, selumetinib, has shown excellent activity in those with RAS pathway mutations. The proapoptotic protein, BIM is pivotal in the induction of cell death by both selumetinib and glucocorticoids, suggesting the potential for synergy. Thus, combination indices for dexamethasone and selumetinib were determined in RAS pathway mutated acute lymphoblastic leukemia primagraft cells in vitro and were indicative of strong synergism (CI <0. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2017.185975DOI Listing
January 2019
6 Reads

Mechanisms of thrombocytopenia in platelet-type Von Willebrand Disease.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Department of Medicine, University of Perugia;

Platelet-type von Willebrand disease is an inherited platelet disorder characterized by thrombocytopenia with large platelets caused by gain-of-function variants in GP1BA leading to enhanced GPIbα von Willebrand factor interaction. GPIbα and von Willebrand factor play a role in megakaryocytopoiesis, thus we aimed to investigate megakaryocyte differentiation and proplatelet-formation in platelet-type von Willebrand disease using megakaryocytes from a patient carrying the Met239Val variant and from mice carrying the Gly233Val variant. Platelet-type von Willebrand disease megakaryocytes bound von Willebrand factor at an early differentiation stage and generated proplatelets with a decreased number of enlarged tips as compared with control megakaryocytes. Read More

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http://dx.doi.org/10.3324/haematol.2018.200378DOI Listing
January 2019
2 Reads

Molecular mechanisms of bleeding disorder-associated GFI1BQ287* mutation and its affected pathways in megakaryocytes and platelets.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Dept. Laboratory Medicine, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherland;

Dominant-negative mutations in transcription factor Growth Factor Independence-1B (GFI1B) like GFI1B-Q287*, cause a bleeding disorder characterized by a plethora of megakaryocyte and platelet abnormalities. The deregulated molecular mechanisms and pathways are unknown. Here we show that both normal and Q287* mutant GFI1B interacted most strongly with the LSD1-RCOR-HDAC co-repressor complex in megakaryoblasts. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.194555DOI Listing
January 2019
5 Reads