Journal of Blood Medicine最新文献

Introduction: Systemic lupus erythematous (SLE) are autoimmune diseases and cerebral venous sinus thrombosis (CVST) is coincidence regarding hypercoagulable condition of both diseases. The presence of both diseases in the same patient is rare, which suggests a relative incompatibility between these diseases.

Case presentation: I report a female case with Systemic Lupus Erythematosus history, aged 27 years, with blurred vision, diplopia, severe headache, numbness and progressive right hemiparesis in 2 weeks. There was narrowing caliber at left transversus and right sigmoid sinus in magnetic resonance venography. She showed improvement in vision, numbness, headache and motor strength in right extremities after receiving pulse dose of corticosteroid for three days.

Conclusion: The distinction between SLE and CVST is a diagnostic challenge for the neurologist, and the presence of both diseases should be considered in patients with clinical neurologic manifestations who present with typical systemic manifestations of SLE and CVST. Neurogenic inflammation can induce disorders of the blood vessel wall (endothelium) that cause hypercoagulability and changes in acute vascular conditions can occur consisting of intraluminal platelet aggregation, thrombosis and also can cause total cerebral thrombotic venous or venular occlusion in SLE patients.

Background: Indonesia is a country with high biodiversity of more than 20,000 plant species, and 35% of them are identified as having health benefits. Moringa oleifera is one plant that almost all of its parts have been used as nutritional supplements and traditional medicines. Moringa leaves contain nutrients, antioxidants, and bioactive substances that have anti-inflammatory, wound healing, and anti-anemia properties.

Purpose: This study aimed to investigate the hematological effect of Moringa leaf powder in male Wistar rats under normal conditions.

Methods: Twenty-four male Wistar rats strain (Rattus norvegicus) 9-10 weeks old and 250-275 grams were divided into four groups (n=6), normal as a control group and three other groups were given Moringa leaf powder at doses 200, 400, and 800 mg/kgBW during 12 weeks. Blood samples at week 12 were administered to determine blood count.

Results: The results of this study showed differences between the various doses of Moringa leaf powder for each hematological profile. These differences were more significant for MCH parameters that indicated a decrease in the D800 group compared with the control group.

Conclusion: In conclusion, this study revealed that the consumption of Moringa leaf powder for 12 weeks did not have a significant change in the hematological profile, except for the MCH value that revealed a modification.

About 75% of persons with hemophilia live in the developing world and do not have access to routine care due to many barriers. There are a lot of challenges associated with hemophilia care in resource-limited settings, ranging from financial to organisational and government commitments. This review discusses some of these challenges and future prospects, while highlighting the important role of the World Federation of Hemophilia in hemophilia patient care. A participative approach involving all stakeholders is key to optimizing care in resource-limited settings.

Purpose: Primary mediastinal large B-cell Lymphoma (PMLBCL) is a rare aggressive lymphoma with unique clinical, pathological, and molecular features. The optimal frontline therapy is subject of ongoing debate. Our study aims to evaluate the outcomes of PMLBCL treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (RCHOP) at King Hussein Cancer Center.

Patients and methods: Adult patients >18 years of age with PMLBCL treated with RCHOP from January 2011 to July 2020 were identified. All demographics, disease and treatment related variables were retrospectively collected. Correlations of clinical and laboratory variables with progression-free survival (PFS) and overall survival (OS) were determined by univariate and multivariate analyses using backward stepwise Cox regression models. The PFS and OS were plotted using Kaplan‒Meier curves.

Results: 49 patients were included with a median age of 29 years. 14 (28.6%) had stage III or IV, 31 (63.3%) had mediastinal bulky disease. International prognostic index (IPI) was 0-1 in 35 (71.4%). Radiotherapy was given to 32 (65.3%) patients. End of treatment (EOT) response was complete (CR) in 32 (65.3%), partial response (PR) in 8 (16.3%) and progressive disease (PD) in 9 (18.4%). Patients who achieved CR at EOT, compared favorably with those who did not in regard to 4-year OS (92.5% vs 26.9%, p=<0.001). Overall objective response to salvage chemotherapies was 26.7%. At a median follow-up of 46 months, 4-year PFS and OS were 60% and 71% respectively. In multivariate analysis, IPI > one correlated with the EOT response (p=0.009), PFS (p=0.004) and OS (p= 0.019).

Conclusion: In PMLBCL, RCHOP chemotherapy backbone in the frontline therapy is suboptimal but can be used in patients with low IPI. Adapting more intensive chemoimmunotherapy regimens may be considered for patients with high IPI. Salvage chemotherapy has limited activity in patients with relapsed or refractory disease.

Introduction: On 24 February 2022, the Russia-Ukraine military conflict unfolded just across the eastern border of the European Union. It made everyone realize how important it is to secure blood supplies to health-care units in the event of an armed conflict. This paper presents the principles of functioning of the Military Blood Donation Service and the Military Center for Blood Donation and Hemotherapy in Poland.

Methods: The study used data collected in the "Military Blood Bank" information processing system and data from annual reports (2010-2021) sent to the Minister of Health of the Republic of Poland. The reports concerned, among others: demographic data on donors, reasons of permanent disqualifications, numbers of complete and incomplete donations, etc.

Results: Since 2005, the number of donors registered in military blood donation centers ranged between 15 and 35 thousand/year. The most dramatic declines in donors were observed in 2010 and 2020. Successful donations accounted for more than 98% of all donations/year (except 2015), and their number varied between 20 and 32 thousand/year. Among the blood donors, men always predominated and the dominant age group (except for 2010) was 25-44 years. The reasons for permanent disqualification have varied over time: their proportions decreased for viral hepatitis and cardiovascular disease, and increased for respiratory and endocrine/metabolic diseases. Due to the COVID-19 pandemic in 2020/2021, these proportions have sometimes been reversed.

Discussion: The Military Blood Donation Service has been functioning in Poland for several decades. It is specialized in supplying blood and blood products to the Armed Forces. Unfortunately, it was not possible to refer to the functioning of similar institutions in other countries. Therefore, when evaluating the functioning of Polish military blood donation, we had to rely on numerical values (eg, number of donors/year, donor profile, etc.), which prove a very good organization of blood donation centers. However, it should be noted that, as in other countries, a more active promotion of blood donation in the media is advisable in order to encourage as many young people as possible to donate blood.

Background: Anemia affects more than a quarter of non-pregnant women over the globe, with Sub-Saharan Africa bearing a disproportionate share. Although the use of family planning is beneficial in reducing anemia, lack of scientific study on anemia among family planning users of reproductive-age women is notable, particularly in the study setting. The purpose of this study was to determine the extent of anemia and associated factors in women who used family planning.

Methods: A cross-sectional multi-centered study was conducted from March 3 to 29, 2019, among 443 non-pregnant reproductive age (15 to 49 years) women receiving family planning services in Ambo town. Sample size was calculated using Epi-info version 7 software. Participants were selected by systematic random sampling technique. Trained data collectors collected data using a structured pretested questionnaire, as well as venous blood and stool samples. Epi-Data and SPSS were used to enter and analyze data. The effect of independent variables on the outcome variable was determined by binary logistic regression analysis with adjusted odds ratio at 95% confidence interval and 5% margin of error. P-value <0.05 was used to declare statistical significance.

Results: This study revealed 28% (95% CI:23.9%, 32.3%) magnitude of anemia. Age of 25-35 years [AOR:2.84, 95% CI:1.74, 4.64], implantable family planning method [AOR: 0.34, 95% CI: 0.12, 0.96], no previous use of family planning [AOR:2.62, 95% CI: 1.62, 4.24], household food insecurity [AOR: 2.04, 95% CI: 1.06, 3.93], parasite infestations [AOR:2.01, 95% CI: 1.12, 3.63], and regular intake of coffee/tea within 30 minutes post meal [AOR:3.85, 95% CI:1.24, 11.92] were independently associated with anemia.

Conclusion: Anemia is a moderate public health concern among reproductive-age women receiving family planning services in the study area. There are missed opportunities to address the anemia burden during family planning services. This study emphasizes the importance of nutritional screening for early detection and targeted interventions for healthcare workers in reducing missed opportunities to prevent and control anemia in vulnerable populations.

Acute basophilic leukemia (ABL) arising from chronic myeloid leukemia (CML) with abundant mast cells (MCs), coexisting with a complex karyotype is rare. Here, we report an 81-year-old man admitted to our hospital with a history of ABL. He was diagnosed with CML in the chronic phase in January 2018, and Imatinib was used at a daily dose of 400mg. Then, transformation to ABL with abundant MCs in the bone marrow and complex karyotypes including 48,XY, trisomy 8 (+8), isochromosome 17(q10) [i(17)(q10)], and derivative chromosome 22 t(9;22) [der(22)t(9;22)] were discovered simultaneously in January 2022. In conclusion, the increased number of MCs in our case is a reminder that they might play an important role in the prognosis of CML and trigger the development of complex karyotypes. Moreover, this is the first case report of ABL arising from CML with abundant MCs, coexisting with 48,XY, +8, i(17)(q10), and der(22)t(9;22), during Imatinib treatment. Further studies are needed to better characterize this rare condition.

Sickle cell disease is caused by an abnormality of the β-globin gene and is characterised by sickling of the red blood cells. Globally, sub-Saharan African countries share the highest burden of the disease. This study aimed at critically reviewing studies focusing on challenges of sickle cell anaemia in sub-Saharan Africa. A literature search was carried out in five major databases. Articles that met the inclusion criteria were included in the bibliometric review and critical analysis. A majority of the studies were undertaken in the West African region (85.5%), followed by Central Africa (9.1%). Very few studies had been undertaken in East Africa (3.6%), whilst the Southern African region had the fewest studies (1.8%). Distribution in relation to country revealed that three quarters of the studies were carried out in Nigeria (74.5%), followed by the Democratic Republic of the Congo (9.1%). According to healthcare settings, a strong majority of the studies were undertaken in tertiary health care facilities (92.7%). Major themes that emerged from the review include interventions, cost of treatment, and knowledge about sickle cell disease. Public health awareness and promotion as well as improving the quality of sickle cell centers for prompt management of patients with sickle cell disorder was identified as a critical strategy towards reducing the burden of the disease in sub-Saharan Africa. To achieve this, governments in countries located in this region need to adopt a proactive strategy in addressing gaps that have been identified in this study, as well as instituting other relevant measures, such as continuous media engagement and public health interventions relating to genetic counselling. Reforms in other areas that can help reduce the disease burden, include training of practitioners and equipping sickle cell disease treatment centers according to World Health Organization specifications.

Severe hemophilia is associated with spontaneous, prolonged and recurrent bleeding. Inadequate prevention and treatment of bleeding can lead to serious morbidity and mortality. Due to the limitations of intravenous clotting factor replacement, including the risk of inhibitory antibodies, innovative novel therapies have been developed that have dramatically changed the landscape of hemophilia therapy. Ribonucleic acid interference (RNAi) has brought the opportunity for multiple strategies to manipulate the hemostatic system and ameliorate the bleeding phenotype in severe bleeding disorders. Fitusiran is a RNAi therapeutic that inhibits the expression of the natural anticoagulant serpin antithrombin. Reduction in antithrombin is known to cause thrombosis if coagulation parameters are otherwise normal and can rebalance hemostasis in severe hemophilia. Reports from late stage clinical trials of fitusiran in hemophilia A and B participants, with and without inhibitory antibodies to exogenous clotting factor, have demonstrated efficacy in preventing bleeding events showing promise for a future "universal" prophylactic treatment of individuals with moderate-severe hemophilia.

The importance of protecting brain function for people with sickle cell disease (SCD) cannot be overstated. SCD is associated with multiple cerebrovascular complications that threaten neurocognitive function and life. Without screening and preventive management, 11% of children at 24% of adults with SCD have ischemic or hemorrhagic strokes. Stroke screening in children with SCD is well-established using transcranial Doppler ultrasound (TCD). TCD velocities above 200 cm/s significantly increase the risk of stroke, which can be prevented using chronic red blood cell (RBC) transfusion. RBC transfusion is also the cornerstone of acute stroke management and secondary stroke prevention. Chronic transfusion requires long-term management of complications like iron overload. Hydroxyurea can replace chronic transfusions for primary stroke prevention in a select group of patients or in populations where chronic transfusions are not feasible. Silent cerebral infarction (SCI) is even more common than stroke, affecting 39% of children and more than 50% of adults with SCD; management of SCI is individualized and includes careful neurocognitive evaluation. Hematopoietic stem cell transplant prevents cerebrovascular complications, despite the short- and long-term risks. Newer disease-modifying agents like voxelotor and crizanlizumab, as well as gene therapy, may treat cerebrovascular complications, but these approaches are investigational.