INTRODUCTION: A minor increase in hemoglobin (Hb) A2 is common in B thalassemia trait in which Hb A2 usually exceeds 3.5%. Other inherited and acquired disorders result in minor increases that do not often exceed 8%. The remarkable increase (>20%) in Hb A2 may refer to other structural variants that also migrate in the Hb A2 window. The aim of this study was to assess the increased Hb A2 levels and their incidence among schoolchildren in Najran city. AIMS: The aim of this study was to assess the increased Hb A2 levels and their incidence among schoolchildren in Najran city. SUBJECTS AND METHODS: This study was conducted on 665 male students of Najran primary schools. All students were subjected to a full history taking and laboratory investigations including a complete blood count, peripheral blood smear examination, measurement of serum iron and ferritin levels, reticulocyte count, and alkaline Hb gel electrophoresis. RESULTS: Four (0.60%) students had highly significant (P = 0.000) increased Hb A2 levels above 20%. Three of them descend to the same tribe. Two cases had mild anemia and the other two cases had normal Hb levels. All cases showed microcytosis and hypochromia with normal serum iron and ferritin levels. CONCLUSION: Alkaline Hb electrophoresis showed highly significant increased Hb A2 levels among school children with microcytosis and hypochromia. Further investigations are required to ensure whether this is an increase of Hb A2 or from other Hb variants that take the same window of Hb A2.

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Patients with hematological malignancies (HM) appear to be at increased risk of acquiring COVID-19 infection and its complications. General measures to protect patients with HM from COVID-19 include limiting exposure of patients to medical environments with the use of telemedicine and virtual clinics, delaying elective diagnostic services, modifying treatment modalities in a manner that reduces the probability of further immune suppression such as shortening the treatment course or prolonging the interval between treatment courses, and growth factor support to reduce the risk of neutropenia. Reducing the threshold for packed RBC and platelet transfusions to mitigate reduced blood product supplies, and a careful attention to drug interactions in patients who get the COVID-19, are also important management measures. Physicians caring for patients with HM need to carefully evaluate each individual patient to optimize therapies, take measures to maximize safety of patients and staff, without significantly compromising outcomes. This paper discusses management of HM during the COVID-19 pandemic.

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INTRODUCTION: During blood storage, red blood cells (RBCs) undergo changes termed “storage lesion” including shape modification and microparticles (MPs) production, as well as oxidative injury to lipids and proteins. OBJECTIVE: We studied the impact of storage on RBCs by quantification of red cell MPs and their potential procoagulant activity (PCA). The antioxidant effect of ascorbic acid (AA) was assessed, as a proposed additive, to improve stored RBC quality. METHODS: Forty randomly selected packed red cell units (filtered and nonfiltered) were sampled weekly over 35 days of storage. Flow cytometric and coagulation tests were performed on MPs isolated from erythrocyte concentrates. RBCs were either in citrate phosphate dextrose adenine-1 (CPDA1) only (standard storage) or CPDA1 with AA (Vitamin C) in three concentrations. RESULTS: Red cell MPs progressively increased over 35 days of storage. Filtration efficiently reduced the production of MPs (P < 0.001). PCA increased significantly on storage assessed by the shortened coagulation time (P < 0.001). A strong inverse correlation between coagulation time and Annexin V positive MPs was found, (r = −0.723, P < 0.001), and the dual-positive population. This highlights the strong correlation between the functional assay (PCA) and the direct quantitative assay (flow cytometry). The red cells fortified with AA demonstrated a reduction in the accumulation of RBC MPs by almost 50% compared to standard storage in CPDA-1 alone in an evident dose-dependent manner. CONCLUSION: We concluded that MPs increase on storage with increased PCA. AA reduced MPs production which can be possible additive for further research in the future

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INTRODUCTION: Sickle cell anemia is associated with intense oxidative stress, and optimal antioxidant levels are essential to prevent continuous oxidant tissue damage. The role of oxidant damage to red blood cells in sickle cell anemia (SCA) has been of interest in recent years, and some available results indicate that sickled red blood cells produce almost twice as much superoxide, hydrogen peroxide, and hydroxyl free radicals compared with normal red cells. To counter the effects of oxidants, red blood cells have evolved a number of self-sustaining activities of antioxidant defense enzymes including but not limited to superoxide dismutase (SOD). This study aimed to measure serum SOD activity in patients SCA patients (as a measure of antioxidant defense) during the crisis and in steady state and to compare this with the activity in HbAA normal controls. SUBJECTS AND METHODS: Eighty-eight participants were recruited into this case control study and comprised 34 HbAA controls, 12 HbSS patients in crisis, and 42 HbSS patients in steady state. Five milliliter of venous blood was collected from each participant and 3 ml was dispensed into plain tubes and centrifuged. The serum was used for the estimation of SOD activity. The remaining 2 ml was used for hemoglobin electrophoresis. RESULTS: SOD levels were highest in the HbAA control group (1.928 ± 0.051 U/mL) followed by the HbSS patients in steady state (1.844 ± 0.118 U/mL) and lowest in the HbSS patients in crisis (1.755 ± 0168 U/mL). This difference was statistically significant with P = 0.001. CONCLUSION: SOD levels are reduced during vaso-occlusive crisis.

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INTRODUCTION: Sickle cell disease (SCD) is autosomal recessive disorder common in the Kingdom of Saudi Arabia (KSA). This is first study in KSA, and middle-east revealing characteristics of pediatric SCD patients admitted to pediatric intensive care unit (PICU). METHODS: Retrospective review of SCD patients admitted to PICU over 6-years-period (2013–2019). Data collected using patient's files and PICU book. Descriptive analysis and percentages presented in tabulated and graphical form. RESULTS: Fifty-eight patients admitted 93 times to PICU, (4.5% of total PICU admission). Most admissions were from station and emergency room (38, 33 admissions respectively). Admissions categorized into emergency and elective (59, 34 admissions respectively). Average length of stay was 1.9 days. Acute chest syndrome (ACS) (n = 28) represented 30% of admissions, followed by cerebrovascular accident (CVA) (18.2%). Five patients (5.37%) admitted for other neurological causes. Twenty-five (26.8%) admitted electively for stroke prevention, 9 (9.6%) admitted postoperatively. Considerable number of exchange transfusion in 65 (69.8%) admissions. Three needed inotropes, five invasive ventilation. Thirteen/58 (22.4%) had previous stroke, 20 (34.4%) had previous ACS, whereas significant number (n = 37, 63.8%) had frequent vaso-occlusive crisis. Mean white blood cell was 18.37 × 10³/μl (emergency group), and 10.4 × 10³/μl (elective group). Mean hemoglobin 7.8 g/dl and 9.67 g/dl in emergency and elective groups, respectively. Seventy percent of patients prescribed hydroxyurea and 27% of patients were on regular transfusion. Mortality rate of SCD in PICU was low (n = 2, 3.4%). CONCLUSION: PICU is one of the essential components of comprehensive health-care services for patients with SCD. Cooperation between intensivists, hematologists, and other health-care providers in addition to the early referral of critically ill SCD patients to PICU leads to better disease outcomes.

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BACKGROUND: Deep venous thrombosis causes morbidity and mortality for hospitalized patients. There are several risk factors for developing deep venous thrombosis including trauma, immobilization, and surgery. Complications of deep venous thrombosis result from the delay in diagnosis and treatment, so prophylaxis is the perfect option to avoid these serious complications, especially for those at risk. AIM: The aim of this is to investigate the impact of DVT protocol on thromboprophylaxis in minimizing the disease burden of under-recognized and preventable pathology. MATERIALS AND METHODS: This is a retrospective descriptive study which used DVT protocol through the Knowledge Translation Committee (KTC). RESULTS: Suboptimal prophylaxis decreased from 47% in 2011 to 6% in 2017, whereas appropriate prophylaxis increased among patients from 45% in 2011 to 89% in 2017. CONCLUSION: There was an increase in offering DVT prophylaxis for hospitalized medical patients as a result of the implementation of DVT protocol through KTC.

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A 61-year-old woman was diagnosed with multiple myeloma (MM) (immunoglobulin G kappa type) in 2012. A bortezomib-based chemotherapy protocol was administered to the patient, and the patient underwent an autologous transplant. During lenalidomide maintenance therapy, she presented with anemia and extramedullary plasmacytomas (EMPs) with no bone marrow infiltration by clonal plasma cells. Her disease was in complete remission after six cycles of the carfilzomib-dexamethasone (KD) protocol. EMPs are seen in MM patients at the time of diagnosis or relapse. There is a challenge related to these patients' treatment schedules, as no consensus or standard treatment protocol has yet been defined. In this article, we present a case regarding the presentation of EMP relapse in a myeloma patient who is in remission after long-term carfilzomib therapy. We demonstrate that a KD treatment may be effective in patients with EMPs.

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Therapy-related secondary hematological changes play a crucial role in determining the life span of an otherwise recovered cancer patient after successful treatment. Hypereosinophilic syndrome (HES) is a potentially life-threatening condition when left unmanaged. The exact etiology of HES, however, remains elusive. We report a case of an adequately treated triple-negative breast cancer patient in remission, who presented with a confirmed diagnosis of HES 2 years after her therapy. On presentation, the blood picture showed leukocytosis of 42.7 × 10⁹/L, where 20% were eosinophils. FIP1-like-1-platelet-derived growth factor receptor-alpha fusion was observed by fluorescence in situ hybridization. The patient was treated with imatinib, according to the World Health Organization guidelines, and full remission was achieved. This is the first case of HES postchemotherapy in a solid malignancy.

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Primary bony anaplastic large-cell lymphoma is quite rare in children but potentially life-threatening lymphoma. Such disease is challenging and often carries a poor prognosis. Here, we report a patient who presented with an aggressive bony lymphoma and failed to respond to conventional chemotherapy; however, he had a robust response to valproic acid, which changed his status from hopeless palliative case to a success story and allowed him to complete his intended chemotherapy protocol.

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