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.

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.

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: 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.

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: Repeated transfusions in thalassemia patients can cause several complications, including alloimmunization and autoimmunization.

Purpose: This study compares the immune response of T follicular helper (Tfh) lymphocytes expressing T-cell immunoreceptor with immunoglobulin and immunoreceptor tyrosine-based inhibitory domains (TIGIT), programmed cell death-1 (PD-1), and interleukin-21 (IL-21) between patients with allo-auto positive and negative thalassemia before and after hemin administration.

Materials and methods: This study used a quasi-experimental pre- and post-test design and was performed between April and November 2021 at the Dr. Soetomo General Academic Hospital in Surabaya, Indonesia. It enrolled 29 patients with allo-auto positive thalassemia and 28 with allo-auto negative, and 9 mL of whole blood (WB) was drawn from each patient. Hemin solution (20 µM) was added to 5 mL of WB, incubated for two hours, processed into peripheral blood mononuclear cells (PBMCs) in RPMI media, and cultured with 5% CO₂ for three days. The 4 mL WB sample was also processed into PBMCs. PBMC cells cultured and without cultured were examined by flow cytometry using a BD FACSCalibur after surface and intracellular staining. Differences in Tfh cells expressing TIGIT, PD-1, and IL-21 between thalassemia groups before and after hemin administration were compared using independent t-tests or Mann-Whitney U-tests (p < 0.05).

Results: Tfh cell expression did not differ between groups before hemin administration and increased after hemin administration. The increase in Tfh cell expression was higher in the allo-auto positive group. TIGIT and PD-1 expression in Tfh cells did not differ between groups, but TIGIT decreased after hemin administration in contrast to PD-1 result. IL-21 expression in Tfh cells did not differ between groups and did not change after hemin administration.

Conclusion: Hemin affected the expression of Tfh cells in both group thalassemia, but there was no difference of Tfh cell expression between the groups.

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.

Purpose: This study aims to investigate the prevalence of inherited thrombophilia in women with recurrent pregnancy loss during the first trimester of pregnancy. The study was assessed the potential role of inherited thrombophilia in recurrent miscarriages and evaluate the consequences of this condition on the reproductive outcomes of affected women.

Material and methods: This study was an analytical descriptive carried out in Khartoum, Sudan. The research comprised 98 controls who had given birth twice or more without experiencing a miscarriage and 120 patients. Each patient had done more than two miscarriages especially when the pregnancy is at its beginning trimester. (APCR), and (PS) were investigated using the clotting approach. There was an assessment of biological activities of (ATIII), (PC), and (PS) for both groups using the chromogenic method.

Results: The average age of the patients was 34, which was higher than the average age of the controls (33.5). The patient group had a much higher rate of multiple miscarriages among the women.: 35 (29.17%), 45 (37.50%), and 40 (33.33%). The incidence of PC deficiencies was determined to be 1.02% (1/98), whereas neither ATIII nor PS deficiencies were seen in the control group (0/98). APCR was more prevalent in the control group (4.10% or 4/98).

Conclusion: Despite contradicting evidence to the contrary in the literature, our findings imply that most miscarriages occur when pregnancy is at the first trimester when a woman is pregnant and they are all caused by thrombophilia.

Chronic myeloid leukemia (CML) is one of the most common leukemias occurring in the adult population. The course of CML is divided into three phases: the chronic phase, the acceleration phase, and the blast phase. Pathophysiology of CML revolves around Philadelphia chromosome that constitutively activate tyrosine kinase through BCR-ABL1 oncoprotein. In the era of tyrosine kinase inhibitors (TKIs), CML patients now have a similar life expectancy to people without CML, and it is now very rare for CML patients to progress to the blast phase. Only a small proportion of CML patients have resistance to TKI, caused by BCR-ABL1 point mutations. CML patients with TKI resistance should be treated with second or third generation TKI, depending on the BCR-ABL1 mutation. Recently, many studies have shown that it is possible for CML patients who achieve a long-term deep molecular response to stop TKIs treatment and maintain remission. This review aimed to provide an overview of CML, including its pathophysiology, clinical manifestations, the role of stem cells, CML treatments, and treatment-free remission.

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.