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08:03 EST 23rd February 2019 | BioPortfolio

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For example view all recent relevant publications on Epigenetics and associated publications and clincial trials.

Showing PubMed Articles 1–25 of 269 from Blood

Successful donor engraftment and repair of the blood brain barrier in cerebral adrenoleukodystrophy.

Adrenoleukodystrophy (ALD) is caused by mutations within the X-linked gene resulting in the inability to transport acylated very long chain fatty acids (VLCFA) into the peroxisome for degradation. VLCFA subsequently accumulate in tissues, including the central nervous system. Up to 40% of boys develop a severe, progressive demyelinating form of ALD, cerebral ALD (cALD), resulting in regions of demyelination observed on brain magnetic resonance imaging (MRI) that are associated with a "garland ring" of gado...

Cdk6 coordinates mutant MPN via NFκB and apoptotic networks.

Over 80% of patients with myeloproliferative neoplasms (MPNs) harbour the acquired somatic mutation. JAK inhibition is not curative and fails to induce a persistent response in most patients, illustrating the need for the development of novel therapeutic approaches. We describe a critical role for CDK6 in MPN evolution. The absence of ameliorates clinical symptoms and prolongs survival. The CDK6 protein interferes with three hallmarks of disease: besides regulating malignant stem cell quiescence, it promo...

The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas.

Primary cutaneous lymphomas are a heterogeneous group of T-cell lymphomas and B-cell lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. In the last decade the 2005 WHO-EORTC consensus classification has served as a golden standard for the diagnosis and classification of these conditions. In September 2018 an updated version of the WHO-EORTC was published in the 4th edition of the WHO classification for Skin Tumours Blue Book. In this classification primar...

Serial transplantation reveals a critical role for endoglin in hematopoietic stem cell quiescence.

TGF-β is well-known for its important function in hematopoietic stem cell (HSC) quiescence. However the molecular mechanism underlining this function remains obscure. Endoglin (Eng), a type III receptor for the TGF-β superfamily, has been shown to selectively mark the long-term HSC, however its necessity in adult HSC is unknown due to embryonic lethality. Using conditional deletion of Eng combined with serial transplantation, here we show that this TGF-β receptor is critical to maintain the HSC pool. Tra...

Durable remissions with obinutuzumab-based chemoimmunotherapy: long-term follow-up of the phase 1b GALTON trial in CLL.

RETRACTION: Mueller S, Meinecke AK, Buchwald S, Eriksson D, and Wilke T. Real-World Adherence to Non-Vitamin K Antagonist Oral Anticoagulants in Patients with Atrial Fibrillation: Results of an International Survey. Blood. 2017;130(Suppl 1):4726.

RETRACTION: Mueller S, Meinecke AK, Buchwald S, Eriksson D, and Wilke T. Does Treatment Satisfaction Influence Adherence to Treatment?: Impact of AF Patients' Treatment Satisfaction on Adherence to Oral Anticoagulation Treatment. Blood. 2017;130(Suppl 1):2142.

Aggressive B-cell lymphomas in patients with myelofibrosis receiving JAK1/2 inhibitor therapy.

Inhibition of Janus-kinase 1/2 (JAK1/2) is a mainstay to treat myeloproliferative neoplasms (MPN). Sporadic observations reported the co-incidence of B-cell non-Hodgkin lymphomas during treatment of MPN with JAK1/2 inhibitors. We assessed 626 MPN patients including 69 with myelofibrosis receiving JAK1/2 inhibitors for lymphoma development. B-cell lymphomas evolved in 4/69 patients (5.8%) upon JAK1/2 inhibition compared to 2/557 (0.36%) with conventional treatment (16-fold increased risk). A similar 15-fold ...

Metformin induces FOXO3-dependent fetal hemoglobin production in human primary erythroid cells.

Induction of red blood cell fetal hemoglobin (HbF, α2γ2) ameliorates the pathophysiology of sickle cell disease (SCD) by reducing the concentration of sickle hemoglobin (HbS, α2β2) to inhibit its polymerization. Hydroxyurea (HU), the only FDA-approved drug for SCD, acts in part by inducing HbF, but is not fully effective, reflecting the need for new therapies. Whole exome sequence analysis of rare genetic variants in SCD patients identified FOXO3 as a candidate regulator of RBC HbF. We validated these g...

Identification of novel mutational drivers reveals oncogene dependencies in multiple myeloma.

Understanding the profile of oncogene and tumor suppressor gene mutations with their interactions and impact on the prognosis of multiple myeloma (MM) can improve the definition of disease subsets and identify pathways important in disease pathobiology. Using integrated genomics of 1,273 newly diagnosed patients with multiple myeloma we identify 63 driver genes, some of which are novel including , , , , and Oncogene mutations are significantly more clonal than tumor suppressor mutations, indicating they ma...

Antiphospholipid antibodies induce thrombosis by PP2A activation via apoER2-Dab2-SHC1 complex formation in endothelium.

In the antiphospholipid syndrome (APS), antiphospholipid antibody (aPL) recognition of β2 glycoprotein (β2GPI) promotes thrombosis, and preclinical studies indicate that this is due to endothelial NO synthase (eNOS) antagonism via apoER2-dependent processes. How apoER2 molecularly links these events is unknown. Here we show that in endothelial cells the apoER2 cytoplasmic tail serves as a scaffold for aPL-induced assembly and activation of the heterotrimeric protein phosphatase PP2A. Dab2 recruitment to t...

Transplant strategies in relapsed/refractory Hodgkin lymphoma.

The majority of patients with Hodgkin Lymphoma (HL) are cured with initial therapy. However, high dose therapy with autologous hematopoietic cell transplant (AHCT) allows for the cure of an additional portion of patients with relapsed or primary refractory disease (rrHL). PET negative complete remission (CR) prior to AHCT is critical for long-term disease control. Several salvage options are available with comparable response rates, and the choice can be dependent of comorbidities and logistics. Radiation t...

Management of relapsed/refractory classical Hodgkin lymphoma in transplant-ineligible patients.

Addition of brentuximab vedotin, a CD30 targeted antibody-drug conjugate, and the PD-1 inhibitors, nivolumab and pembrolizumab, to the armamentarium for transplant-ineligible relapsed/refractory classical Hodgkin lymphoma has resulted in improved outcomes, including the potential for cure in a small minority of patients. For patients who have failed prior transplant or are unsuitable for dose-intense approaches based on age or comorbidities, an individualized approach with sequential use of single agents su...

Introduction to a review series on Hodgkin Lymphoma: Change is here.

Optimizing therapy in advanced stage Hodgkin lymphoma.

The treatment of Hodgkin Lymphoma has evolved continuously since the introduction of extended-field radiotherapy in the 1960s to involved-field then involved-node radiotherapy, multi-agent chemotherapy, combined chemo-radiotherapy, risk-adapted and response-adapted modulation, and most recently, introduction of antibody-drug conjugates and immune checkpoint-blocking antibodies. These changes have translated into progressively increasing cure rates, so that 10-year survival figures now exceed 80%, compared t...

Early-stage classical Hodgkin lymphoma.

With defined chemo- and radiotherapy and risk-adapted treatment, early-stage classical Hodgkin lymphoma (HL) has become curable in the majority of patients. A major current goal is hence to reduce treatment-related toxicity while maintaining long-term disease control. Patients with early-stage favorable disease, i.e. limited stage without risk factors (RFs), are frequently treated with two cycles of doxorubicin, bleomycin, vinblastine and dacarbazine (2xABVD) followed by 20Gy involved-field or -site radioth...

Biology of classical Hodgkin lymphoma: implications for prognosis and novel therapies.

Hodgkin lymphoma is considered a prime example of treatment success with cure rates exceeding 80% using modern combined modality therapies. However, especially in adolescents and young adults, treatment-related toxicity and long-term morbidity still represent persistent challenges. Moreover, outcomes in patients with relapsed or refractory disease remain unfavorable in the era of high-dose chemotherapy and stem cell transplantation. Hence, there is a high demand for novel and innovative alternative treatmen...

How I treat myelodysplastic syndromes of childhood.

Pediatric myelodysplastic syndromes (MDS) are a heterogeneous group of clonal disorders with an annual incidence of 1-4 cases/million, accounting for less than 5% of childhood hematological malignancies. MDS in children often occur in the context of inherited bone marrow failure syndromes, this representing a peculiarity of myelodysplasia diagnosed in the pediatric age. Moreover, germline syndromes predisposing individuals to develop MDS/acute myeloid leukemia have recently been identified, such as those ca...

Allele-selective RUNX1 binding regulates P1 blood group status by transcriptional control of.

P1 and Pare glycosphingolipid antigens synthesized by the-encoded α1,4-galactosyltransferase, using paragloboside and lactosylceramide as acceptor substrates, respectively. In addition to the compatibility aspects of these histo-blood group molecules, both constitute receptors for multiple microbes and toxins. Presence or absence of P1 antigen on erythrocytes determines the common P(P1+P+) and P(P1-P+) phenotypes.transcript levels are higher in Pindividuals and SNPs in non-coding regions of, particularly r...

CD38 pretargeted RIT of B-cell tumors.

ENLightening YEATS in antagonizing polycomb repression.

Myosin IIa signal von Willebrand factor release.

Syphilitic lymphadenitis with abscess formation involving cervical lymph nodes.

Interference of hyperleukocytosis in red blood cell count.

Wong RSM, Saleh MN, Khelif A, et al. Safety and efficacy of long-term treatment of chronic/persistent ITP with eltrombopag: final results of the EXTEND study.. 2017;130(23):2527-2536.

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