Journal of Blood Medicine最新文献

Background: Severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection has been a global threat since the end of 2019. Although the main clinical manifestation of coronavirus disease 2019 (COVID-19) is respiratory, its range of clinical manifestation is extensive and may include various systems, including hematological disorders, such as lymphopenia, thrombotic events, thrombocytopenia and immune thrombocytopenic purpura (ITP). The present case was the first one that aimed to raise awareness of ITP induced by COVID-19 in patients undergoing maintenance hemodialysis.

Case presentation: This is the case of a 75-year-old Asian woman who was diagnosed COVID-19 positive 15 days before attending our Emergency Department on January 19th, 2023, with a three-day history of severe bleeding symptoms, including gastrointestinal, mucosal bleeding, epistaxis, and the platelet count of 5×10⁹/L. She suffered from end-stage kidney disease due to autosomal dominant polycystic kidney disease and has received thrice-weekly maintenance hemodialysis (MHD) since 2012. Platelet count recovery was observed after 45 days of combined treatment with corticosteroids, intravenous immunoglobulin, thrombopoietin receptor agonists, and rituximab. The count of platelets rose to 180×10⁹/L after four dosages of Rituximab.

Conclusion: In brief, SARS-CoV-2 infection might trigger the onset of ITP. To our knowledge, this is the first case with severe and refractory ITP secondary to COVID-19 in MHD patients and no guidelines were able to be referred on the therapy. Nephrologists must be concerned with clinical characteristics, diagnostic flowcharts, and therapy for SARS-CoV-2-induced ITP.

Objective: To determine the fears and myths related to blood donation in future health care workers.

Study design: Cross-sectional study.

Place and duration of study: This study was carried out from October to December 2022 at the National University of Medical Sciences (NUMS), Rawalpindi, Pakistan. Donors were selected according to the, WHO recommended, Safe Blood Transfusion Program of Pakistan criteria.

Results: In total, 411 participants were included in the study. The individuals were 21-24 years of age, with a mean age of 21 years. In our study, females dominated (232/411); the remaining 179 were males. Out of the total 411, 145 participants had previously donated blood while the other 266 had never donated blood. Our study analyzed both of these groups. The most common symptoms experienced by blood donors were dizziness, post-donation weakness, and bodily aches and pains. Most non-donors feared problems related to their general health (42.3%) and developing infections (12.7%). P-value was 0.002, which reveals a significant association between fears and intention to donate blood.

Conclusion: These results suggest that fears and concerns related to blood donation play a leading role in forecasting donors' attitudes and intentions. Motivation leads to inspiration and potential donors can be motivated by addressing their fear.

Aim: Anemia, primarily due to iron deficiency, is a key risk factor in both elective and emergency surgeries. Immediate preoperative treatment with ferric carboxymaltose (FCM) in anemic patients can reduce the need for transfusions and the length of hospital stay, thereby optimizing surgical outcomes. The objective of this study was to assess the effectiveness and describe the use of administering intravenous FCM prior to elective scheduled surgery for patients diagnosed with anemia.

Methods: Multicenter, retrospective cohort study that encompassed patients aged 18 years and older who underwent surgery between January 2017 and December 2018. Demographic variables, dose scheme, baseline and perioperative haemoglobin (Hb), transfusion requirements, and admission days were collected. The primary endpoints were the response rate and effectiveness of FCM, defined as the proportion of patients with Hb preoperative levels of ≥13 g/dL. A patient response was deemed to occur when Hb level increased by 1 g/dL or more. The secondary endpoints were the appropriateness of FCM dose, transfusion requirement rate, and length of hospital stay.

Results: 446 patients (55.2% women, median age 69 IQR:52-78 years) were included. The median total dose of FCM administered was 1000 mg over a span of 5 day (IQR: 0-16) days before surgery. 62.8% of patients received lower doses, 24.9% had an INCREASE of Hb ≥ 1 g/dL, 11.6% had Hb ≥ 13 g/dL and 21.3% required blood transfusions, with a mean of 0.73 units transfused. The length of the hospital stay was 12 days (IQR:6-23).

Conclusion: Low percentage of patients achieved a hemoglobin level of 13 g/dL or experienced an increase in hemoglobin of 1 g/dL or more following the administration of FCM, indicating the low effectiveness of FCM in treating perioperative anaemia in our surgical patients. There is underdosing of FCM and insufficient time between FCM administration and surgery in most patients. Both transfused and non-transfused patients show similar Hb increases, while those receiving a standard 1000 mg dose of FCM experience shorter hospital stays compared to those receiving 500 mg, and patients with more transfusions have longer hospital stays.

Background: Despite its long-term history, haematopoietic stem cell transplantation (HSCT) still faces challenges in several countries under development and especially in the private health sector.

Objectives design and methods: In this retrospective analysis, we present our experience and the results of 48 adult patients who underwent HSCT (autologous 37, allogeneic 9) at a private-sector hospital in Abu Dhabi, United Arab Emirates (UAE). The main indications were multiple myeloma and acute myeloid leukaemia in the autologous and allogeneic setting, respectively.

Results: All patients successfully engrafted, and after a median follow-up of 6 (range: 1-11) months, 42 patients are alive (36 autografted and 6 allografted). The 1-year overall survival rates were 97% and 62% for autografted and allografted patients, respectively, while 4 patients died within 100 days post-transplant from treatment-related causes (1 patient from the autografted group and 3 patients from the allografted group).

Conclusion: Our data confirm that HSCT is a feasible, safe, and effective treatment approach, offering high success rates to UAE patients with haematological malignant diseases.

Objective: Hemolysis is the most severe change that occurs in stored blood and can cause severe consequences in patients after transfusion. This study examines the potential role of IgG and complement, exampled by C4, in the hemolysis of stored CPDA-1 blood under poor storage conditions in low-income countries.

Methods: The study was performed on 30 whole blood units (250 mL) drawn from convenience healthy volunteer donors with CPDA-1 anticoagulant and stored at 2-6 °C for 35 days. Each well-mixed blood bag was sampled at 0, 7, 21 and 35 days and examined for CBC, plasma hemoglobin, hemolysis percent and determination of IgG and C4.

Results: The plasma hemoglobin level and hemolysis percent increased continuously to reach 1.56 g/dl and 7.05% at the end of storage time. Hemolysis increased alongside the mean IgG concentration that was increased significantly from day 0 of storage (7.68±1.75 g/L) and peaked on day 7 (11.55±1.57 g/L), then declined to reach 8.33±2.09 g/L on day 35. Also, the mean concentration of C4 increased from day 0 of storage (0.15±0.06 g/L) to a peaked on day 21 (0.18±0.04) then declined on day 35 (0.17±0.06 g/L). The coordinated action of IgG and C4 is reflected by the positive correlation of their delta changes (r=0.616, p<0.0001).

Conclusion: Elevated hemolysis percent in whole CPDA-1 stored blood in Yemen was accompanied by initial increase of IgG and C4 followed by final decline, which indicate their activation and consumption during hemolysis. Further studies for other hemolysis markers and analyses will give a full idea about that.

Objective: To identify a suitable approach for blood irradiation other than the commonly used water medium and to study the impact of different algorithm dose computations.

Methods: Water is the commonly used medium for blood irradiation. In this study computed tomography scans were taken with locally made blood irradiation phantoms other than water, by using air, rice powder and thermocole using parallel beam for 25 Gy. Plans were recalculated for different algorithms such as collapsed cone (CC), Monte Carlo (MC) and pencil beam (PB). The dose-volume parameters and measured doses were collected and analyzed for each medium and algorithm.

Findings: The monitor unit (MU) for rice powder and water are close (2461±57 and 2469±61, respectively), with a maximum dose of 28.0±1.8 and 28.0±1.9 Gy. The PB algorithm resulted in lower monitor unit values regardless of the medium used, generating values of 2418, 2406, 2382, and 2362 for water, rice powder, air, and Thermocol, respectively. A significant increase in dose was observed irrespective of the medium used when the MC algorithm was employed, with a maximum of 30.26 Gy in rice powder; a smaller dose was used when the CC algorithm was employed, with 26.3 Gy in water medium. The average maximum doses of all groups were equal using the one-way Anova statistical test. Regarding the impact of field size, rice powder appears to have consistent doses across various field sizes, with slight increases as field size grows, which is similar to water.

Novelty/applications: While water is the conventional medium, this study highlights the potential benefits of rice powder, such as eliminating the risks associated with bubble formation and water spillage, which can lead to equipment malfunction and safety hazards. Although previous studies have explored rice powder as a bolus and tissue-equivalent material, this study uniquely applies this knowledge to blood irradiation, an area where rice powder has not been thoroughly investigated.

Sickle cell disease (SCD), the most common autosomal recessive genetic disorder, affects the hemoglobin (Hb) chains in human red blood cells. It is caused by mutations in the β-globin genes, leading to the production of hemoglobin S, which results in the formation of sickle-shaped red blood cells (RBCs). These abnormal cells cause hemolysis, endothelial damage, and small vessel occlusion, leading to both acute and long-term complications. According to the World Health Organization's 2008 estimates, SCD affects approximately 2.28 per 1000 individuals globally. Despite this high prevalence, therapeutic advancements have been slow. For many years, the only FDA-approved medications for managing SCD complications were hydroxyurea and deferiprone. However, recent years have seen the approval of several new therapies, including L-glutamine (2017), voxelotor and crizanlizumab (2019), as well as exagamglogene autotemcel (Casgevy) and lovotibeglogene autotemcel (Lyfgenia) (2023). These treatments have proven effective in managing both the acute and chronic effects of SCD, including hemolytic anemia, chronic pain, stroke, vaso-occlusive crises, and multiple organ damage syndromes. This review explores the mechanisms of action, practical considerations, and side effects of these emerging therapies, drawing from a comprehensive search of databases such as PubMed, Medline, and Cochrane.

Advanced systemic mastocytosis (AdvSM) is a rare hematologic malignancy with organ damage and compromised life expectancy arising from organ accumulation of neoplastic mast cells. Identification of the gain-of-function KITD816V in the majority of cases has accelerated pharmaceutical development culminating with the development of selective KIT inhibitors such as avapritinib. While the advent of these therapies has improved the quality and quantity of life in patients with AdvSM, current challenges remain in the management of this disease. In this review, we summarize the present and future therapeutics landscape of AdvSM, highlighting the development of novel KIT inhibitors including elenestinib and bezuclastinib. We also explore the continued role of additional treatment modalities including allogeneic stem cell transplantation before discussing unresolved clinical challenges in the management of AdvSM.

Background: The application of rituximab has significantly enhanced the overall survival rates in patients with diffuse large B-cell lymphoma (DLBCL). Regrettably, a significant number of patients still progress to relapse/refractory DLBCL (rrDLBCL).

Methods: Herein, we employed targeted sequencing of 55 genes to investigate if gene mutations could predict the progression to rrDLBCL. Additionally, we compared the mutation profiles at the time of DLBCL diagnosis with those found in rrDLBCL cases.

Results: Our findings highlighted significantly elevated mutation frequencies of TP53, MEF2B and CD58 in diagnostic biopsies from patients who progressed to relapse or refractory disease, with CD58 mutations exclusively observed in the rrDLBCL group. In assessing the predictive power of mutation profiles for treatment responses in primary DLBCL patients, we found that the frequency of CARD11 mutations was substantially higher in non-response group as compared with those who responded to immunochemotherapy. In addition, we revealed mutations in HIST2H2AB, BCL2, NRXN3, FOXO1, HIST1H1C, LYN and TBL1XR1 genes were only detected in initial diagnostic biopsies, mutations in the EBF1 gene were solely detected in the rrDLBCL patients.

Conclusion: Collectively, this study elucidates some of the genetic mechanisms contributing to the progression of rrDLBCL and suggests that the presence of CD58 mutations might serve as a powerful predictive marker for relapse/refractory outcomes in primary DLBCL patients.

Background: Methemoglobin is an altered state of hemoglobin where iron in hemoglobin is oxidized and incapable of binding oxygen; leading to complications such as cyanosis, dyspnea, headache, and heart failure. Methemoglobinemia can be congenital or acquired. Congenital methemoglobinemia is a rare disease and its worldwide incidence is unclear. We recently encountered the first documented case of congenital methemoglobinemia at our institution, necessitating perioperative care.

Case presentation: In the present case, a 22-year-old man with congenital methemoglobinemia underwent general anesthesia for dental extraction. The surgeon was informed to avoid local anesthetics and oxygenation was performed with FiO₂ of 1.0. Arterial blood gas analysis showed a PH of 7.337, PaO₂ of 302 mm Hg, PaCO₂ of 44 mm Hg, oxyhemoglobin level of 63.4%, and methemoglobin level of 37.8%. The patient had a stable course. No methylene blue therapy was required, although cyanosis was observed during surgery.

Conclusion: In summary, though rare, congenital methemoglobinemia poses fatal risks during surgery. Its management involves preoperative recognition and optimization, oxygenation status, multidisciplinary care, avoiding precipitating or oxidizing agents, discussing treatment options, maintaining cardiopulmonary stability, and ensuring perioperative safety measures with the medical team.