Subscribe: pubmed: Eur J Hem[jour]
http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=0rmp-ilgffswpASPNHcEa4stRoF-4Pw81Cv5l8E75TL
Added By: Feedage Forager Feedage Grade B rated
Language: English
Tags:
article protected  cell  emp  etv  germline etv  hsct  oncology  patients  pediatric patients  pediatric  pres  protected rights  stem cell 
Rate this Feed
Rate this feedRate this feedRate this feedRate this feedRate this feed
Rate this feed 1 starRate this feed 2 starRate this feed 3 starRate this feed 4 starRate this feed 5 star

Comments (0)

Feed Details and Statistics Feed Statistics
Preview: pubmed: Eur J Hem[jour]

pubmed: Eur J Hem[jour]



NCBI: db=pubmed; Term="European Journal of Haematology"[jour]



 



Detection of a new heterozygous germline ETV6 mutation in a case with hyperdiploid acute lymphoblastic leukemia (ALL).
Related Articles

Detection of a new heterozygous germline ETV6 mutation in a case with hyperdiploid acute lymphoblastic leukemia (ALL).

Eur J Haematol. 2017 Oct 16;:

Authors: Duployez N, Abou Chahla W, Lejeune S, Marceau-Renaut A, Letizia G, Boyer T, Geffroy S, Peyrouze P, Grardel N, Nelken B, Michel G, Bertrand Y, Preudhomme C

Abstract
ETV6 is a target of recurrent aberrations in sporadic and familial acute lymphoblastic leukemia (ALL). Here, we report on a new pedigree with a germline ETV6 mutation in which the index patient and his father developed high-hyperdiploid (HeH)-ALL and polycythemia vera at age 13 and 51 respectively. The index patient achieved durable complete remission without transplantation but had persistent moderate thrombocytopenia without bleeding tendency. In order to determine the prevalence of ETV6 alterations in HeH-ALL, we screened 81 unrelated subjects with HeH-ALL by single nucleotide polymorphism-array and high-throughput sequencing for the ETV6 gene. Overall, ETV6 microdeletions and mutations were identified in 9% of cases, all of which were somatic and considered as secondary events. Apart from the index patient, no germline ETV6 aberration was identified. Finally, we reviewed the literature for ETV6 germline aberrations and predispositions to ALL. This article is protected by copyright. All rights reserved.

PMID: 29034503 [PubMed - as supplied by publisher]




A survey on hematology-oncology pediatric AIEOP centres: the challenge of posterior reversible encephalopathy syndrome.
Related Articles

A survey on hematology-oncology pediatric AIEOP centres: the challenge of posterior reversible encephalopathy syndrome.

Eur J Haematol. 2017 Oct 15;:

Authors: Zama D, Gasperini P, Berger M, Petris M, De Pasquale MD, Cesaro S, Guerzoni ME, Mastrodicasa E, Savina F, Ziino O, Kiren V, Muggeo P, Mura RM, Melchionda F, Zanazzo GA, behalf of Supportive Therapy Working Group of Italian pediatric haematology and oncology association (AIEOP).

Abstract
OBJECTIVES: Posterior reversible encephalopathy syndrome (PRES) is one of the most common neurological complications in hematology-oncology pediatric patients. Despite an increasingly recognized occurrence, no clear consensus exists regarding how best to manage the syndrome, because most cases of PRES have reported in single case reports or small series. Aim of this paper is to identify incidence, clinical features, management and outcome of PRES in a large series of hematology-oncology pediatric patients METHODS: The cases of PRES occurred in twelve centres of the Italian Association of Pediatric Haematology and Oncology were reported RESULTS: 124 cases of PRES in 112 pediatric patients were recorded with an incidence of 2.1% and 4.7% respectively in acute lymphoblastic leukemia in first complete remission and hematopoietic stem cell transplantation (HSCT). The majority of cases occurred after a cycle of chemotherapy rather than after stem cell transplant. PRES after chemotherapy significantly differs from that after HSCT for diagnosis, time of presentation, risk factors, management and outcome CONCLUSIONS: This study demonstrates that PRES is a common neurological complication and occurring preferentially in course of induction treatment of some hematologic malignancies, as ALL and after HSCT. It also highlights great clinical differences in the management and outcome in patients with PRES occurring after chemotherapy or after HSCT This article is protected by copyright. All rights reserved.

PMID: 29032616 [PubMed - as supplied by publisher]




Erythroblast macrophage protein (Emp): Past, Present and Future.
Related Articles

Erythroblast macrophage protein (Emp): Past, Present and Future.

Eur J Haematol. 2017 Oct 15;:

Authors: Javan GT, Salhotra A, Finley SJ, Soni S

Abstract
This review is a journey of the landmark protein Erythroblast macrophage protein (Emp) discovered in 1994, and it walks chronologically through the progress that has been made in understanding the biological function of this protein. Historically, Emp was the first identified cell attachment molecule and is expressed in both erythroblasts and macrophages and mediate their attachments to form erythroblastic islands. The absence of Emp erythroblasts shows defects in differentiation and enucleation. Emp-deficient macrophages display immature morphology characterized by small size, round shapes, and the lack of cytoplasmic projections. Although the primary sequence of Emp has already been determined and its role in both erythroid and macrophage development is well established, there are major gaps in the understanding of its function at the molecular level. Recent studies had implicated its importance in actin cytoskeleton remodeling and cell migration but the molecular mechanisms are still enigmatic. Previous studies have also demonstrated that down-regulation of Emp affects the expression of MAPK1 and AKT-1 resulting in abnormal cell motility. In this review, we summarize the proposed function of Emp based on previous studies, present scenarios, and its plausible future in translational research. This article is protected by copyright. All rights reserved.

PMID: 29032607 [PubMed - as supplied by publisher]




Infection surveillance in pediatric hematopoietic stem cell transplantation recipients.
Related Articles

Infection surveillance in pediatric hematopoietic stem cell transplantation recipients.

Eur J Haematol. 2017 Oct 15;:

Authors: Teixeira DC, Diniz LMO, Mourão PHO, Kakehashi FM, de Macedo AV, Duani H, Clemente WT, de Sá Rodrigues KE, de Castro Romanelli RM

Abstract
OBJECTIVE: Describe the profile of reported Healthcare-Associated Infections (HAIs) in pediatric patients submitted to hematopoietic stem cell transplantation (HSCT) at a reference center.
METHODS: Retrospective cohort of pediatric patients who were submitted to HSCT from 2008 to 2016. The criteria for HAI were based on those established by the National Healthcare Safety Network. Data were collected by active surveillance performed daily by professionals. This study was approved by the Institutional Research Ethics Committee.
RESULTS: A total of 86 HSCTs were performed in 81 patients younger than 18 years of age (median, 10 years). Of these, 69 (85%) were males. Aplastic anemia and leukemia were the main diagnoses. A total of 140 HAIs were diagnosed with an incidence density of 28.2 infections/1.000 patient-days. The most common HAI was Laboratory Confirmed Bloodstream Infection (46), the majority of which was reported to be central venous catheter-associated (43). Gram-negative bacteria were the most prevalent microorganisms (58.5%). Almost all the infections occurred until 30 days after transplantation, and 17 deaths were observed within 180 days after the procedure.
CONCLUSION: Active surveillance of HAIs in HSCT children allowed the evaluation of the incidence and profile of HAIs, which is essential for the healthcare of these patients. This article is protected by copyright. All rights reserved.

PMID: 29032585 [PubMed - as supplied by publisher]