Hematology Reports 2019-07-23T09:55:01+02:00 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> Concurrent juvenile myelomonocytic leukemia with thalassemia in a case with Plasmodium knowlesi infection from Sabah, Malaysian Borneo 2019-07-23T09:54:52+02:00 Mimi Azreen Abdullah Saleh mohammed Abdullah Subbiah Vijay Kumar Mohammad Zahirul Hoque <p>A 3-year-old male child was presented with worsening abdominal pain, abdominal distension, lethargy, pallor and hepatosplenomegaly. The patient had multiple outpatient visits in the past and was treated with oral antibiotics, oral anthelmintic agents, albeit with minimal benefit. The patient also had non-neutropenic pyrexia spikes and oral ulcers. The patient was an adopted child; hence details about his biological parents’ previous history were unclear. Differential diagnosis of Chronic Myelomonocytic Leukemia (CMML), Juvenile Myelomonocytic Leukemia (JMML), Gaucher’s disease, Thalassemia and discrete pancreatic pathology was considered. Haemoglobin electrophoresis was indicative of thalassemia. Also, molecular detection method by polymerase chain reaction (PCR) confirms a concurrent infection with <em>Plasmodium knowlesi malaria. The </em>BCR-ABL&nbsp;fusion gene was found to be negative. Correlating with peripheral monocytosis, Bone Marrow Aspiration (BMA) and trephine biopsy with blasts only 3-4% and hepatosplenomegaly, a diagnosis of Juvenile Myelomonocytic Leukemia was established. We present a rare phenomenon with an overlap of signs and symptoms between JMML, underlying thalassemia, and <em>Plasmodium knowlesi</em>, posing a diagnostic challenge to physicians.</p> <p>&nbsp;</p> 2019-06-24T14:25:58+02:00 ##submission.copyrightStatement## Patient compliance with deep vein thrombosis prophylaxis after total hip and total knee arthroplasty 2019-07-23T09:54:54+02:00 Daniel H. Wiznia Nishwant Swami Jenny Nguyen Eric Musonza Chris Lynch David Gibson Richard Pelker <p>Even though the Centers for Medicare and Medicaid Services is penalizing hospitals for readmissions, and postoperative prophylaxis has demonstrated reduced complications associated with deep vein thrombosis (DVT), few studies have examined patient compliance with (DVT) prophylaxis at home. A survey querying DVT prophylaxis management and adherence was administered to patients who were within the one to three-month postoperative period after a total knee or total hip replacement. A total of 103 patients completed the survey. A considerable number of patients (17.0%) were non-adherent to DVT prophylaxis. Patients had a lower understanding of the side effects of their DVT prophylaxis, with 30% responding that they had a poor to simple understanding. There is a high rate of non-compliance and there is a poor understanding of complications related to venous thromboembolism prophylaxis. As the population undergoing arthroplasties grows, this study demonstrates the importance of investigating the role of medication adherence in the rate of postoperative DVT.</p> 2019-06-17T15:49:39+02:00 ##submission.copyrightStatement## B-cell acute lymphoblastic leukemia as a secondary malignancy following diffuse large B-cell lymphoma 2019-07-23T09:54:56+02:00 Daria Gaut Anthony Bejjani Joshua Sasine Gary Schiller <p>Secondary acute lymphoblastic leukemia (ALL) is a rare disease that has not been well characterized compared with secondary myelodysplastic syndrome or secondary acute myeloid leukemia. We present a report of two patients who developed ALL following complete remission of diffuse large B-cell lymphoma (DLBCL). The first case is more consistent with a therapy-related ALL as a PCR analysis of bone marrow aspirate revealed a distinct clone and the mixed-lineage leukemia gene rearrangement, commonly associated with exposure to topoisomerase II inhibitors. The second case is more consistent with clonal evolution given positive MYC and BCL2 fusion signals in the original diagnosis of DLBCL and the secondary ALL.</p> 2019-06-14T12:37:37+02:00 ##submission.copyrightStatement## Health status and quality of life in patients with severe hemophilia A: A cross-sectional survey 2019-07-23T09:54:58+02:00 Majid Davari Zahra Gharibnaseri Roya Ravanbod Abolfazl Sadeghi <p>Among different groups of hemophiliacs, those suffering from Severe Hemophilia A (SHA) are most vulnerable to the complications of the disease. This study investigated the Health-Related Quality of Life (HR-QoL) among adult patients with SHA. A cross-sectional study was designed to gather demographic and clinical information from adult patients with SHA. Patients with inhibitors were excluded. The remaining were asked to complete the HR-QoL questionnaire after being examined for joint health using the Hemophilia Joint HealthScore (HJHS). The HR-QoL and joint conditions were measured in 38 patients. The mean EQ-5D value scores were 0.46 (SD=0.23) while the mean Visual Analogous Scale score was 50 (SD=18.7). The clinical examination of patients indicated that the HJHS were as follows: eight patients had a score of 55-75, 12 patients had a score of 40-55, 7 of them (25-40) and 11 patients had a score of 10-25. The results obtained from this study showed that HR-QoL in hemophilia patients was considerably low. Pain, anxiety/depression, and motion limitations were the main causes of the disutility for these patients respectively.</p> 2019-06-14T12:30:19+02:00 ##submission.copyrightStatement## Implications of intrachromosomal amplification of chromosome 21 on outcome in pediatric acute lymphoblastic leukemia: Does it affect our patients too? 2019-07-23T09:55:01+02:00 Suleimman Al-Sweedan Rahaf Altahan <p>Intrachromosomal amplification (iAMP) of chromosome 21 entity is associated with a dismal outcome in B cell Acute Lymphoblastic Leukemia (B-ALL). This cytogenetic abnormality is caused by a novel mechanism; breakage-fusion-bridge cycles followed by chromothripsis along with major gross rearrangements in chromosome 21.Charts of B-ALL diagnosed at King Faisal Specialist Hospital and Research Center between 2005 and 2015 were reviewed.iAMP is a rare entity occurring at around 2.4% of all pediatrics B-ALL. No statistically significant difference was found among patients with iAMP21, patients with extra copies of 21 and other patients with B-ALL. The reported adverse prognostic effect of iAMP21 could be due to other coexistent adverse factors, including older age at the time of diagnosis. The most common associated abnormality in our population in addition to the hyperdiploidy was ETV6/RUNX1.</p> 2019-06-14T12:27:15+02:00 ##submission.copyrightStatement##