PubMed Journal Database | European journal of haematology 
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Showing PubMed Articles 1–25 of 59 from European journal of haematology
A 27-year-old Burmese male initially presented to the health department for a routine vaccination approximately one month after arriving in the United States as a refugee. He had no significant past medical history, but was found to have abdominal distension and discomfort before being referred to the clinic. He did not have fever, nausea, vomiting or other symptoms. Physical examination was remarkable for a markedly enlarged spleen that was tender to palpation. No jaundice or skeletal abnormalities were no...
BACKGROUND: Serum soluble tumor necrosis factor receptor 2 (sTNFR2) concentration predicted the clinical outcome of patients with aggressive non-Hodgkin's lymphoma including diffuse large B-cell lymphoma (DLBCL) treated with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) in our previous study. However, after rituximab (R) was introduced in clinical practice, R-CHOP replaced CHOP as the standard therapy for DLBCL. PATIENTS AND METHODS: In this study, we re-evaluated the prognostic signif...
No consecutive analysis of BAALC and WT1 expressions associated with core-binding factor AML (CBF-AML) from diagnosis to hematopoietic stem cell transplantation (HSCT) has yet been reported. We investigated BAALC and WT1 expressions using a method of real-time quantitative polymerase chain reaction (RQ-PCR) at diagnosis, after induction chemotherapy, at pre- and post-HSCT period in 45 consecutive patients (t(8;21) (n=28), inv(16) (n=17)), who received HSCT as a post-remission treatment. BAALC and WT1 RQ-PCR...
Renal diseases in haemophilic patients: pathogenesis and clinical management.
Haemophilia A and B are genetic X-linked bleeding disorders, caused by mutations in genes encoding factor VIII and IX, respectively. Clinical manifestations of haemophilia are spontaneous haemorrhage or acute bleeding caused by minor trauma, resulting in severe functional consequences that can culminate in a debilitating arthropathy. Life expectancy and quality of life of haemophilic patients have dramatically improved over the last years, mainly for new therapeutic options and the awareness to the risk of...
OBJECTIVES: A host of medical, socio-demographic, and psychological factors that affect bone marrow transplantation (BMT) patients' quality of life (QOL) has been identified but due to the methodological diversity of the studies, the findings have been contradictory. The aim of this study was to examine the influence of somatic status, social characteristics, and psychological symptoms on QOL in BMT patients. METHODS: The study had a cross-sectional design. QOL was evaluated using the Hungarian version of t...
OBJECTIVES: Decorin is a stromal-produced small leucine-rich proteoglycan known to attenuate tumour pro-survival, migration, proliferation and angiogenic signalling pathways. Recent studies have shown that decorin interacts with the hepatocyte growth factor (HGF) receptor c-Met, a potential key pathway in multiple myeloma (MM). METHODS: Decorin levels in paired peripheral blood and bone marrow plasma samples from healthy volunteers (HV) (n=23), and patients with monoclonal gammopathy of undetermined signifi...
It is with great interest that we have watched the results of the studies done in mice suggesting that bortezomib treatment could be deleterious to bone health in children. These concerns have been extrapolated from a recent report (1) which suggests that bortezomib can decrease bone growth velocity and bone matrix deposition in young mice, an effect mediated by induction of chondrocyte apoptosis. In this study, young mice were treated with 1 mg/kg of bortezomib (equivalent to 25 mg/m(2) in children). While...
The mechanisms by which parathyroid hormone (PTH) produces anemia, are unclear. Parathyroid hormone secretion is regulated by the extracellular Ca(2+) -sensing receptor. We investigated the effects of ablating PTH on hematological indices and erythrocytes volume regulation in wild-type, PTH-null and Ca(2+) -sensing receptor-null/PTH-null mice. The erythrocyte parameters were measured in whole mouse blood and volume regulatory systems were determined by plasma membrane K(+) fluxes and osmotic fragility was m...
Extramedullary diseae in patients with Acute Myeloid Leukemia assessed by (18)F-FDG PET.
BACKGROUND: Prevalence of extramedullary disease (EMD) in acute myeloid leukemia (AML) at the time of diagnosis is unknown. Previous estimates ranges from 2.5% to 30.5% and is usually based on clinical examination. This may cause an under diagnosis of EMD since not all extramedullary manifestations are easily detectable. Few recent studies have used positron emission tomography (PET) scans for diagnosing EMD in AML-patients. METHOD: During a 9 month period newly diagnosed AML-patients who were candidates fo...
There is a growing body of evidences that acquired chromosomal abnormalities in bone marrow (BM) cells are associated with clinical manifestations of myelodysplastic syndrome (MDS). However, to our knowledge, there are no reports that describe the association between chromosomal abnormalities in MDS and graft-versus-host disease (GVHD) after allogeneic stem cell transplantation (allo-SCT). Here we describe two MDS cases with trisomy 8 and der(1;7)(q10;p10), who developed severe GVHD after allo-SCT. We analy...
OBJECTIVES: The categorization of T-cell acute lymphoblastic leukemia (T-ALL) into four subgroups according to the degree of thymic differentiation was proposed in 1995, and this categorization scheme has been described in the World Health Organization classification 4(th) edition with minor changes. The aim of this study is to explore the clinical significance of leukemia cell differentiation stages in patients with T-ALL. METHODS: We analyzed 36 adult T-ALL patients, including six patients (17%) in pro-T...
Candidemia detected on direct blood smears.
A one-year old boy with a history of Hirschsprung disease underwent an elective sigma resection by transverse infraumbilical laparotomy. A subclavian vein catheterization for parenteral nutrition was performed simultaneously. Antibiotic therapy with ceftriaxone and metronidazole was begun. © 2013 John Wiley & Sons A/S.
ALK-positive large B-cell lymphoma presenting with jejunal intussusception.
No association between the STAT5b rs6503691 (C>T) SNP and myeloproliferative neoplasms.
Polyctyhemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and chronic myeloid leukemia (CML) are the four classical myeloproliferative neoplasms (MPN), characterized by specific somatic activating mutations (eg JAK2 V617F and the BCR-ABL fusion). The STAT (Signal Transducer Activator of Transcription) family comprises several transcription factors with a pleiotropic expression. They have important roles in cells metabolism, growth and division. © 2012 John Wiley & Sons A/S.
The present study describes a retrospective analysis on the transplant outcome of 56 consecutive myelodysplastic syndrome (MDS) patients according to their response to hypomethylating agents (HMA). While 2-year disease-free survival (DFS) of patients who transformed to acute myeloid leukemia (n = 12) was 25%, that of the remaining MDS patients according to response to HMA was 73.1%, 68.1%, 50.0%, and 20.8% in G-COR (group of continuous response, n = 19), G-NoC (group of no change, n = 15), G-LOR (group of l...
Molecular Cloning of IGλ Rearrangements using Long Distance Inverse-PCR (LDI-PCR).
OBJECTIVES: Malignant cells of mature B-cell origin show tumor-specific clonal immunoglobulin gene (IG) rearrangements, including V(D)J recombinations, nucleotide mutations or translocations. Rapid molecular cloning of the breakpoint sequence by long-distance inverse PCR (LDI-PCR) has so far been applied to rearrangements targeted to IGH joining, IGH switch and IGκ regions. We tended to apply LDI-PCR method for cloning of IGλ rearrangements. METHODS: To identify which IGλ isotype segment was rearranged,...
Predictors of Hemoglobin Response to Eculizumab Therapy in Paroxysmal Nocturnal Hemoglobinuria.
BACKGROUND: Paroxysmal nocturnal hemoglobinuria is a clonal, hematopoietic stem cell disorder that manifests with hemolytic anemia and bone marrow failure. Eculizumab has been shown to improve anemia, decrease intravascular hemolysis and reduce the risk of thrombosis. DESIGN AND METHODS: This is a retrospective, single-center study of patients treated with Eculizumab and categorized according to response criteria. Complete response was defined as transfusion independence with normal hemoglobin for age/sex,...
Spindle-shaped diffuse large B-cell lymphoma (Sp-DLBCL) has been recognized as a rare morphologic variant of DLBCL. However, the biological processes that contribute to the specific features of Sp-DLBCL remain poorly understood. In this study, a combined immunophenotypic and genetic analysis was performed in 10 Sp-DLBCL. First, we investigated several unique markers for anaplasia (CD30, ALK, CD68, and EBER-ISH), mesenchyma (SMA, desmin, and vimentin), and B-cell differentiation (CD10, BCL6, and MUM1). We al...
Constitutive activation of STAT5 (by phosphorylation) has been identified in a number of malignancies, including acute myeloid leukemia (AML). Objectives: We investigated whether the level of phosphorylated STAT5 (pSTAT5) expression correlates with clinical outcome in AML. Methods: Adult patients with newly diagnosed AML receiving induction chemotherapy and with an available diagnostic bone marrow were evaluated. Results: Forty-two percent had pSTAT5 expression > 0 on immunohistochemical analysis of fixed b...
INTRODUCTION: Since the early 1990s, recombinant human clotting factor VIII (rhFVIII) produced in hamster cells has been available for haemophilia A treatment. However, the post-translational modifications of these proteins are not identical to those of native human FVIII, which may lead to immunogenic reactions and the development of inhibitors against rhFVIII. For the first time, rhFVIII produced in a human host cell line is available. AIM: We describe here the establishment of the first human production...
Neutrophil-specific glycoprotein CD177 is expressed on a subset of human neutrophils and has been shown to be a counter-receptor for platelet endothelial cell adhesion molecule-1 (PECAM-1, CD31). Previous studies have demonstrated that the interaction of CD177 with endothelial PECAM-1 supports neutrophil transendothelial migration resulting in preferential transmigration of the CD177-expressing neutrophil subset. Since PECAM-1 is also abundantly expressed on platelets, we addressed a follow-up suggestion th...
BACKGROUND: Few studies have evaluated the risk of pregnancy-related adverse events in asymptomatic relatives of probands for VTE and factor V Leiden or the G20210A variant. The antepartum management of this population ranges from antepartum anticoagulation therapy to clinical surveillance. OBJECTIVE: To evaluate the risk of placenta-mediated pregnancy complications and pregnancy-related VTE in VTE-asymptomatic families of probands with VTE and who are heterozygous carriers of either factor V Leiden or PT-G...
Low-Risk Identification In Multiple Myeloma Using A New 14-Gene Model.
Identifying the best gene expression pattern associated with low-risk disease in newly diagnosed multiple myeloma (MM) patients is important to direct clinical treatments. The Multiple Myeloma Survival Index14 (MMSI14) was developed from GEP data sets of 22 normal plasma cells (NPC), 5 MM cell lines (MMCL), 44 monoclonal gammopathy of undetermined significance (MGUS), and 351 newly diagnosed MM patients. R/bioconductor and siggenes package were used to obtain heatmap, boxplot and histogram which results wer...
Therapy-related acute myeloid leukemia (t-AML) is a clinical syndrome occurring as a complication after cytotoxic and/or radiation therapy. The incidence of t-AML after acute promyelocytic leukemia ATRA and anthracycline-based therapy is rather low. However, because of the high remission rates and long-term overall survival achieved with current APL treatments, late complications related to anti-leukemic therapy should be taken into account, giving priority to efficacy agents with the lowest potential of le...
Plasma cell myeloma with intracytoplasmic crystalloid inclusions, mimicking a Niemann-Pick disease.
The patient was a 50-year-old male who was incidentally found to have a serum monoclonal protein spike while being evaluated for donation of blood components. Serum immunoglobulin profiling showed 1,534 mg/dL of IgG. Immunofixation electrophoresis demonstrated a monoclonal serum IgG kappa protein. © 2012 John Wiley & Sons A/S.
