Hematology Reports https://www.pagepress.org/journals/index.php/hr <p><strong>Hematology Reports</strong> is an Open Access, online-only, peer-reviewed journal that considers mainly original articles, case reports and reviews on all aspects of prevention, diagnosis and management of disorders of the blood, as well as related molecular and cell biology, genetics, pathophysiology, epidemiology and controlled trials. Manuscripts on laboratory and clinical aspects of blood-related conditions are welcome, as well as research on the treatment of blood diseases. Occasional issues and supplements publish an in-depth clinical and biological analysis of particular types of blood diseases. The journal also reviews important recent developments in the biology and treatment of malignant diseases, and highlights promising new directions.</p> en-US <p><strong>PAGEPress</strong> has chosen to apply the&nbsp;<a href="http://creativecommons.org/licenses/by-nc/4.0/" target="_blank" rel="noopener"><strong>Creative Commons Attribution NonCommercial 4.0 International License</strong></a>&nbsp;(CC BY-NC 4.0) to all manuscripts to be published.<br><br> An Open Access Publication is one that meets the following two conditions:</p> <ol> <li>the author(s) and copyright holder(s) grant(s) to all users a free, irrevocable, worldwide, perpetual right of access to, and a license to copy, use, distribute, transmit and display the work publicly and to make and distribute derivative works, in any digital medium for any responsible purpose, subject to proper attribution of authorship, as well as the right to make small numbers of printed copies for their personal use.</li> <li>a complete version of the work and all supplemental materials, including a copy of the permission as stated above, in a suitable standard electronic format is deposited immediately upon initial publication in at least one online repository that is supported by an academic institution, scholarly society, government agency, or other well-established organization that seeks to enable open access, unrestricted distribution, interoperability, and long-term archiving.</li> </ol> <p>Authors who publish with this journal agree to the following terms:</p> <ol> <li>Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.</li> <li>Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.</li> <li>Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.</li> </ol> emanuela.fusinato@pagepress.org (Emanuela Fusinato) tiziano.taccini@pagepress.org (Tiziano Taccini) Tue, 19 Feb 2019 07:47:26 +0100 OJS 3.1.1.4 http://blogs.law.harvard.edu/tech/rss 60 Relapsing thrombotic thrombocytopenic purpura with low ADAMTS13 antigen levels: An indication for splenectomy? https://www.pagepress.org/journals/index.php/hr/article/view/7904 <p>With more recent modalities of immunosuppression, splenectomy is now rarely considered in refractory/relapsed thrombotic thrombocytopenic purpura (TTP). However, the surgical approach had shown convincing evidences of high efficacy in the pre-rituximab era and therefore may still represent a lifesaving option in selected challenging cases. To define the characteristics of subjects who may benefit from splenectomy may ease clinical decision making. In this paper we describe the clinical and laboratory data of 2 multiple relapsing TTP cases who successfully underwent splenectomy in the pre-rituximab era. Whereas high anti-ADAMTS13 antibody titre and low ADAMTS13 activity never correlated with remission and relapse, a drop in the ADAMTS13 antigen level was always associated with the acute phase, whereas levels consistently returned to normal following splenectomy, heralding long term remission. Splenectomy may therefore be considered in refractory TTP cases associated with increased ADAMTS13 antigen clearance, irrespective of persistence of inhibitory antibodies.</p> Anna Maria Lombardi, Irene Di Pasquale, Maria Antonietta Businaro, Irene Cortella, Silvia Ferrari, Fabrizio Fabris, Fabrizio Vianello ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc/4.0 https://www.pagepress.org/journals/index.php/hr/article/view/7904 Tue, 19 Mar 2019 08:37:24 +0100 MPL Y252H and MPL F126fs mutations in essential thrombocythemia: Case series and review of literature https://www.pagepress.org/journals/index.php/hr/article/view/7868 <p>Essential thrombocythemia (ET) is a clonal bone marrow disease, characterized by increased production of platelets along with other clinical and bone marrow findings. Most patients with ET will have a somatic mutation in one of the known gene locations of <em>JAK2, CALR</em> , or <em>MPL</em> that can upregulate the JAK-STAT pathway. <em>MPL</em> mutation is present in 5% of cases with the most common mutations being <em>W515L</em> and <em>W515K.</em> In this report we describe 2 cases of patients with clinical and laboratory picture of ET. One patient carried <em>MPLY252H</em> mutation which is previously unreported in the adult population but has been shown to be a gain-of-function mutation. The other patient carried <em>MPL F126fs</em> mutation which is not known to be of clinical importance and has not been previously reported.</p> Ahmed G. Elsayed, Aeesha Ranavaya, Muhammad Omer Jamil ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc/4.0 https://www.pagepress.org/journals/index.php/hr/article/view/7868 Tue, 12 Mar 2019 08:55:45 +0100 Studying anemia of chronic disease and iron deficiency in patients with rheumatoid arthritis by iron status and circulating hepcidin https://www.pagepress.org/journals/index.php/hr/article/view/7708 <p>Anemia of chronic disease (ACD) and iron deficiency anemia (IDA) are the two most important types of anemia in rheumatoid arthritis (RA). Functional iron deficiency in ACD can be attributed to overexpression of the main iron regulatory hormone hepcidin leading to diversion of iron from the circulation into storage sites resulting in iron-restricted erythropoiesis. The aim is to investigate the role of circulating hepcidin and to uncover the frequency of IDA in RA. The study included 51 patients with RA. Complete blood counts, serum iron, total iron binding capacity, ferritin, and hepcidin- 25 were assessed. ACD was found in 37.3% of patients, IDA in 11.8%, and combined (ACD/IDA) in 17.6%. Serum hepcidin was higher in ACD than in control and the other groups (P≤0.001). It was strongly and positively correlated with ferritin (P&lt;0.001), while hemoglobin, serum iron, and total iron binding capacity were negatively correlated with hepcidin (P=0.016, 0.022 and &lt;0.001, respectively). High serum hepcidin was significantly associated with ACD in RA. IDA alone or combined with ACD was encountered in about a third of patients.</p> Warqaa Khalaf, Haithem Ahmed Al-Rubaie, Sami Shihab ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc/4.0 https://www.pagepress.org/journals/index.php/hr/article/view/7708 Tue, 12 Mar 2019 08:39:35 +0100 A unique case of durable complete remission after salvage with azacitidine and DLI for high risk flt-3 positive acute myeloid leukemia, following relapse 18 months post allogeneic stem cell transplant https://www.pagepress.org/journals/index.php/hr/article/view/7800 <p>A unique case of primary refractory FLT3-itd mutated acute myeloid leukemia in an elderly patient, who achieved completed morphological remission, and FLT3-itd negativity, following 9 cycles of azacitadine in combination with escalating doses of donor lymphocyte infusions following relapse 18 months post reduced intensity HLAA mismatch Campath conditioning allogeneic stem cell transplant.</p> Claire Horgan, Alexandros Kanellopoulos, Shankara Paneesha, Bhuvan Kishore, Richard Lovell, Emmanouil Nikolousis ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc/4.0 https://www.pagepress.org/journals/index.php/hr/article/view/7800 Mon, 25 Feb 2019 10:17:59 +0100 Patterns of prescribing hydroxyurea for sickle cell disease patients from a central hospital, Saudi Arabia https://www.pagepress.org/journals/index.php/hr/article/view/7860 <p>Sickle cell disease (SCD) is a group of inherited disorders of hemoglobin synthesis. It is prevalent in different parts of the world, including the Kingdom of Saudi Arabia. The disease is associated with multiple acute and chronic life-threatening complications. Hydroxyurea (HU) is an effective preventive medication; its use has resulted in decreased morbidity and mortality. However, practice variability, including underutilization of HU, has been reported. No local publication has addressed this issue. The aim of this work is to consider the pattern of HU prescription for SCD patients. This is a retrospective study included patients seen in the outpatient clinics in a central hospital. Cases of medications unavailability or patient refusal to take the drug were not included. A total of 152 patients were included, of them 118 were prescribed HU and 34 were not. In 133 (87.5%) patients, the physician’s decision was appropriate. Inappropriate decisions including both under prescription and, to much lesser extent, over utilization had been demonstrated in 19 (12.5%) cases. Impact of raising the healthcare providers’ awareness and improving compliance with the updated SCD management recommendations and guidelines deserve further studying. In our local experience, although the majority of HU prescriptions were appropriate, both under prescription and to a lesser extent, overutilization was demonstrated.</p> Assem A. Elghazaly, Adel A. Aljatham, Abdulaziz M. Khan, Rasha M. Elneil, S. Zubair S. Jafar, Salah A. Elwishy, Omsalma E. Ahmed, Aamer M. Bughnah, Mamoun H. Ibrahim ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc/4.0 https://www.pagepress.org/journals/index.php/hr/article/view/7860 Tue, 19 Feb 2019 08:48:18 +0100