Significance of thrombocytopenia in patients with primary and postessential thrombocythemia/polycythemia vera myelofibrosis
We present clinical characteristics and outcome of patients with either PMF, PPV‐MF, or PET‐MF, and thrombocytopenia who presented to our institution between 1984 and 2015. Of 1269 patients (877 PMF, 212 PPV‐MF, 180 PET‐MF), 11% and 14% had platelets either <50 × 109/L or between 50‐100 × 109/L, respectively. Patients with platelets <50 × 109/L were most anemic and transfusion dependent, had highest blast count and unfavorable karyotype. In general, their overall and leukemia‐free survival was the shortest with median time of 15 and 13 months, respectively; with incidence of acute leukemia almos...
Source: European Journal of Haematology - January 17, 2018 Category: Hematology Authors: Lucia Masarova, Ahmad Alhuraiji, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Jorge Cortes, Sherry Pierce, Hagop Kantarjian, Srdan Verstovsek Tags: ORIGINAL ARTICLE Source Type: research

Thrombopoietin receptor agonists in patients with persistent or chronic immune thrombocytopenia
ConclusionsOur study provides some initial data on the potential benefits of the treatment with TPO‐RAs that may allow splenectomy to be safely deferred for prolonged periods. More research is needed to evaluate the impact of the treatment with TPO‐RAs prior to splenectomy. (Source: European Journal of Haematology)
Source: European Journal of Haematology - January 15, 2018 Category: Hematology Authors: Jacopo Agnelli Giacchello, Federica Valeri, Mario Boccadoro, Alessandra Borchiellini Tags: ORIGINAL ARTICLE Source Type: research

Outcome following second allogeneic hematopoietic cell transplantation: A single ‐center experience
ConclusionSecond HCT may offer long‐term survival particularly to patients with good performance status who relapse post‐first HCT. (Source: European Journal of Haematology)
Source: European Journal of Haematology - January 11, 2018 Category: Hematology Authors: Hassan A. Aljasem, Hans A. Messner, Jeffrey H. Lipton, Dennis Dong Hwan Kim, Auro Viswabandya, Santhosh Thyagu, Uday Deotare, Fotios V. Michelis Tags: ORIGINAL ARTICLE Source Type: research

DNA and RNA binding by the Wilms' tumour gene 1 (WT1) protein +KTS and −KTS isoforms—From initial observations to recent global genomic analyses
Abstract The Wilms' tumour gene 1 protein (WT1) is a zinc finger transcription factor found indispensable for foetal development. WT1 has also been implicated in the development of tumours in several organ systems, including acute myeloid leukaemia (AML). Four main WT1 isoforms come from 2 alternative splice events. One alternative splice results in the inclusion or exclusion of 3 amino acids, KTS, between zinc fingers 3 and 4 in the WT1 protein. The KTS insert has been extensively investigated due to the functional implications for DNA and RNA binding. In this review, we provide an overview of the research into the isofor...
Source: European Journal of Haematology - January 10, 2018 Category: Hematology Authors: Tove Ullmark, Giorgia Montano, Urban Gullberg Tags: REVIEW ARTICLE Source Type: research

Chimerism and gene therapy — Lessons learned from non‐conditioned murine bone marrow transplantation models
European Journal of Haematology,Volume 100, Issue 4, Page 372-382, April 2018. (Source: European Journal of Haematology)
Source: European Journal of Haematology - January 9, 2018 Category: Hematology Source Type: research

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European Journal of Haematology, Ahead of Print. (Source: European Journal of Haematology)
Source: European Journal of Haematology - January 8, 2018 Category: Hematology Source Type: research

Two novel unstable hemoglobin variants due to in ‐frame deletions of key amino acids in the β‐globin chain
This article is protected by copyright. All rights reserved. (Source: European Journal of Haematology)
Source: European Journal of Haematology - January 1, 2018 Category: Hematology Authors: Karen Gabriela Scheps, Marcia Anah í Hasenahuer, Gustavo Parisi, Héctor M. Targovnik, Eliana García, Ernesto Samuel Veber, Renée Crisp, Graciela Elena, Viviana Varela, María Silvina Fornasari Tags: Original Article Source Type: research

Chimerism and Gene Therapy – Lessons Learned from Non‐Conditioned Murine Bone Marrow Transplantation Models
ConclusionBy extrapolating our murine data, and data from some previous studies to a human non‐conditioned autologous CD34+ HSPC transplantation setting, we conclude that approximately 44 million CD34+ HSPCs would be needed to achieve 20% donor chimerism in a 70 kg human, which could serve as a starting point for the future use of HSCPs in gene and cell therapy.This article is protected by copyright. All rights reserved. (Source: European Journal of Haematology)
Source: European Journal of Haematology - January 1, 2018 Category: Hematology Authors: Katerina Jazbec, Mojca Je ž, Boštjan Smrekar, Simona Miceska, Jasmina‐Živa Rožman, Urban Švajger, Janja Završnik, Tadej Malovrh, Primož Rožman Tags: Original Article Source Type: research

Case Report: Treatment of light ‐chain amyloidosis with daratumumab monotherapy in two patients
Abstract Immunoglobulin light‐chain amyloidosis (AL) affects multiple organs, most prominently the kidney and the heart. Renal and cardiac impairment are both associated with poor prognosis and most patients die as a consequence of renal or cardiac failure. Monoclonal antibodies such as daratumumab (human IgG1 anti‐CD38) and elotuzumab (anti‐SLAMF7) have shown promising efficacy for the treatment of relapsed and refractory multiple myeloma. In this case report we show 2 patients with severe AL, one with severe heart failure and one with heart and renal failure, undergoing treatment with daratumumab. Both patients sho...
Source: European Journal of Haematology - January 1, 2018 Category: Hematology Authors: Charlotte Gran, G östa Gahrton, Evren Alici, Hareth Nahi Tags: CASE REPORT Source Type: research

Omitting cytogenetic assessment from routine treatment response monitoring in CML is safe
ConclusionWe conclude that it is safe to omit routine cytogenetics for response assessment during treatment and to only use molecular monitoring, in order to prevent ambiguous classifications, reduce costs and reduce the need for invasive bone marrow sampling. Cytogenetic re‐assessment should still be performed when molecular response is suboptimal.This article is protected by copyright. All rights reserved. (Source: European Journal of Haematology)
Source: European Journal of Haematology - December 30, 2017 Category: Hematology Authors: Inge G.P. Geelen, Noortje Thielen, Jeroen J.W.M. Janssen, Mels Hoogendoorn, Tanja J.A. Roosma, Peter J.M. Valk, Otto Visser, Jan J. Cornelissen, Peter E. Westerweel Tags: Original Article Source Type: research

Disease and treatment characteristics of polycythaemia vera patients in Belgium: results from a scientific survey
ConclusionThis survey provides better insight on the characteristics of Belgian PV patients and currently used treatment strategies. It shows that 232 (67.6%) PV patients continue to receive hydroxyurea (HU) despite being potentially HU‐resistant.This article is protected by copyright. All rights reserved. (Source: European Journal of Haematology)
Source: European Journal of Haematology - December 29, 2017 Category: Hematology Authors: Timothy Devos, Yves Beguin, Lucien Noens, Koen Van Eygen, Pierre Zach ée, Philippe Mineur, Laurent Knoops, Chantal Doyen, Koen Theunissen, Fleur Samantha Benghiat, Michael Reusens, Wim Pluymers Tags: Original Article Source Type: research

Systematic review of infectious events with the BTK inhibitor ibrutinib in the treatment of haematologic malignancies
ConclusionsIbrutinib use requires prudent consideration of the impacts on host immunity. We identified a high rate of serious adverse infectious events within prospective clinical trials. Data suggest a role of both BTK and ITK inhibition for the increased events. There was considerable variability in the reporting of adverse events between trials, journals, and conference reports.This article is protected by copyright. All rights reserved. (Source: European Journal of Haematology)
Source: European Journal of Haematology - December 29, 2017 Category: Hematology Authors: Benjamin F. Tillman, James M. Pauff, Gowri Satyanarayana, Mahsa Talbott, Jeremy L. Warner Tags: Review Article Source Type: research

Omitting cytogenetic assessment from routine treatment response monitoring in chronic myeloid leukemia is safe
European Journal of Haematology,Volume 100, Issue 4, Page 367-371, April 2018. (Source: European Journal of Haematology)
Source: European Journal of Haematology - December 29, 2017 Category: Hematology Source Type: research

Disease and treatment characteristics of polycythemia vera patients in Belgium: Results from a scientific survey
European Journal of Haematology,Volume 100, Issue 4, Page 361-366, April 2018. (Source: European Journal of Haematology)
Source: European Journal of Haematology - December 29, 2017 Category: Hematology Source Type: research