Hematology Reports 2019-03-24T09:35:50+01:00 Emanuela Fusinato Open Journal Systems <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> Relapsing thrombotic thrombocytopenic purpura with low ADAMTS13 antigen levels: An indication for splenectomy? 2019-03-24T09:35:42+01:00 Anna Maria Lombardi Irene Di Pasquale Maria Antonietta Businaro Irene Cortella Silvia Ferrari Fabrizio Fabris Fabrizio Vianello <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> 2019-03-19T08:37:24+01:00 ##submission.copyrightStatement## MPL Y252H and MPL F126fs mutations in essential thrombocythemia: Case series and review of literature 2019-03-24T09:35:44+01:00 Ahmed G. Elsayed Aeesha Ranavaya Muhammad Omer Jamil <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> 2019-03-12T08:55:45+01:00 ##submission.copyrightStatement## Studying anemia of chronic disease and iron deficiency in patients with rheumatoid arthritis by iron status and circulating hepcidin 2019-03-24T09:35:46+01:00 Warqaa Khalaf Haithem Ahmed Al-Rubaie Sami Shihab <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> 2019-03-12T08:39:35+01:00 ##submission.copyrightStatement## 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 2019-03-24T09:35:48+01:00 Claire Horgan Alexandros Kanellopoulos Shankara Paneesha Bhuvan Kishore Richard Lovell Emmanouil Nikolousis <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> 2019-02-25T10:17:59+01:00 ##submission.copyrightStatement## Patterns of prescribing hydroxyurea for sickle cell disease patients from a central hospital, Saudi Arabia 2019-03-24T09:35:50+01:00 Assem A. Elghazaly Adel A. Aljatham Abdulaziz M. Khan Rasha M. Elneil S. Zubair S. Jafar Salah A. Elwishy DR_SALAH_LORD@YAHOO.COM Omsalma E. Ahmed Aamer M. Bughnah Mamoun H. Ibrahim <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> 2019-02-19T08:48:18+01:00 ##submission.copyrightStatement##