Journal of Blood Medicine最新文献

Introduction: COVID-19 infection has brought new challenges to the treatment of adult patients with immune thrombocytopenia (ITP). In adult ITP patients, there have been no relevant reports exploring the incidence, clinical characteristics, and risk factors of platelet elevation after COVID-19 infection.

Materials and methods: A total of 66 patients with previously diagnosed ITP from December 2022 to February 2023 in a single-center were collected and analyzed for this real-world clinical retrospective observational study.

Results: In the platelet count increased group (n = 19), 13 patients (68.4%) were using thrombopoietin receptor agonists (TPO-RA) treatment at the time of COVID-19 infection; the median platelet count was 52 (2-207) ×10⁹/L at the last visit before infection and 108 (19-453) ×10⁹/L at the first visit after infection. In the platelet count stable group (n = 19) and platelet count decreased group (n = 28), 9 (47.4%) and 8 (28.6%) patients were using TPO-RA at the time of infection, respectively. ITP patients treated with TPO-RA had a significantly higher risk of increased platelet count than those not treated with TPO-RA at the time of infection (platelet count increased group vs platelet count decreased group: OR: 5.745, p = 0.009; platelet count increased group vs the non-increased group: OR: 3.616, p = 0.031). In the platelet count increased group, the median platelet count at 6 months post-infection was 67 (14-235) × 10⁹/L, which was significantly higher than the platelet level at the last visit before infection (p = 0.040).

Conclusion: This study showed that some adult ITP patients had an increase in platelet count after COVID-19 infection, and this phenomenon was strongly associated with the use of TPO-RA at the time of infection. Although no thrombotic events were observed in this study, it reminds clinicians that they should be alert to the possibility of thrombotic events in the long-term management of adult ITP patients during the COVID-19 pandemic.

Background: Sickle cell disease is an inherited blood disorder which can lead to severe complications, particularly in the cardiovascular and respiratory systems, potentially resulting in arrhythmias, pulmonary hypertension (PH), and cardiomegaly. This study aims to investigate the risk of PH and arrhythmias in adult SCD patients.

Methods: Retrospective analysis of medical records from King Abdulaziz University Hospital (KAUH) for patients with SCD aged 15 and above between 2009 and 2021. The study included 517 patients, with echocardiograms and electrocardiograms assessed according to the European Society of Cardiology/the European Respiratory Society (ESC/ERS) guidelines for categorizing PH risk (low, moderate, high) and detecting arrhythmias. Data analysis employed the Statistical Package for the Social Sciences (SPSS), utilizing quantitative and qualitative data representation. Multivariate logistic regression identified independent risk factors with odds ratios at a 95% confidence interval (CI).

Results: Among participants, 50.3% were male, with a total sample average age of 34.45 ± 9.28 years. Results indicated that 1.4% of patients experienced arrhythmias, 3.7% had a moderate PH risk, and 3.3% were classified as high PH risk. Logistic regression revealed significant independent risk factors for PH and arrhythmia in patients with SCD, with chronic kidney disease (CKD) carrying the highest odds (26.4 times higher odds of PH and 15.36 times higher odds of arrhythmias).

Conclusion: Patients with SCD are at risk for developing PH and various arrhythmias but are often underdiagnosed. Key risk factors for PH included CKD, liver cirrhosis, and pre-existing cardiac conditions. Arrhythmias were significantly associated with CKD and pre-existing cardiac conditions. To mitigate these risks, we recommend involving a multidisciplinary healthcare team in the care of adult patients with SCD. Future prospective studies are advised for early detection of PH and arrhythmias in hemoglobinopathy patients, potentially reducing mortality.

Purpose: The state of Montana encompasses and defines rural health care as it is known in the United States (US) today. This vast area is punctuated by pockets of health care availability with varying access to blood products for transfusion. Furthermore, timely transport is frequently challenged by weather that may limit air transportation options, resulting in multiple hours in ground transport to definitive care.

Patients and methods: The Montana State Trauma Care Committee (MT-STCC) developed the Montana Interfacility Blood Network (MT-IBN) to ensure blood availability in geographically distanced cases where patients may otherwise not survive. The index case that led to the formal development of the MT-IBN is described, followed by a second case illustrating the IBN process.

Results: This process and development manuscript details the innovative efforts of MT-STCC to develop this fledgling idea unique to rural US health care. We review guidelines that have been developed to define broad aspects of the MT-IBN including the reason to share resources, proper packaging, paperwork necessary for transfer, and how to provide resources directly to the patient. Finally, we describe implementation within the state.

Conclusion: The MT-IBN was developed by MT-STCC to facilitate the hand-off of lifesaving blood to patients being transported by ground to definitive care in Montana without having to stop at an intermediary facility. This has already led to lives saved in areas that are limited in blood availability due to rurality.

Background: Newborn anemia is among the most common hematological problems and it can cause asymptomatic or severe to acute life-threatening events. It leads to impairment in brain maturation and development, tissue hypoxia, and stunted growth and then arrested growth if left untreated. The prevalence of anemia among newborns ranges from 23.4-66% in sub-Saharan Africa. But, there is limited information in Ethiopia regarding the prevalence of newborn anemia and its risk factors. Therefore, this study aimed to determine the prevalence of newborn anemia and its associated factors at Jimma Medical Center (JMC), South-west Ethiopia.

Methods: A hospital-based cross-sectional study design was implemented from January 14 to February 28, 2021, involving 288 full-term newborns by employing consecutive convenient sampling technique for study participant selection. Socio-demographic data and other associated factors were collected through interviews and a review of medical records by a structured questionnaire. Three mL umbilical cord blood samples from each newborn were collected and analyzed for a complete blood count by an automated hematological analyzer. Data were entered into Epi Data version 3.1 and exported to Statistical Package for Social Science version 20 for analysis. Binary logistic regression were used to identify the predictors of newborn anemia.

Results: The overall prevalence of anemia among newborns was 26.4%; of them, 65.8%, 25%, and 9.2% were mild, moderate, and severe anemia types, respectively. Maternal vegetable consumption habit (AOR = 0.26, 95% CI: 0.11, 0.62) and maternal anemia (AOR = 0.34, 95% CI: 0.17, 0.69) were significantly associated with anemia in newborns.

Conclusion: In general, newborn anemia in this study was a moderate public health problem. Based on this study, early screening of anemia among newborns may reduce further complications. Prevention of maternal anemia during pregnancy by improving their nutritional status especially vegetable consumption had a positive impact on reducing anemia among newborns.

Rosai-Dorfman Disease (RDD) is a rare non-Langerhans histiocytosis, usually self-limited and presenting with massive, painless, bilateral cervical lymphadenopathy, with or without constitutional symptoms. Extranodal disease is frequently present, and may happen in the absence of lymph node involvement, symptomatology and differential diagnosis will depend on the site affected and fatal cases may occur. The authors present two cases of Rosai-Dorfman disease (RDD), diagnosed through immunohistochemistry, with different progressions, one with complete remission and one culminating in death, highlighting the variety of presentations and the diagnostic difficulty. RDD is a rare condition with clinical presentations similar to several diseases, and should be considered in the differential diagnosis of lymphadenopathy with extranodal lesions.

Introduction: Purified factor IX (FIX) concentrate (IMMUNINE^®, Takeda Manufacturing Austria AG, Vienna, Austria) is indicated for the treatment and prophylaxis of bleeding episodes in patients with congenital hemophilia B. Data on the use of purified FIX concentrate in patients ≤6 years old with congenital hemophilia B are limited.

Aim: Document real-world clinical experience with purified FIX concentrate in routine practice for pediatric patients with hemophilia B.

Methods: This prospective post-authorization safety surveillance study enrolled patients ≤6 years old with moderate or severe hemophilia B (baseline FIX ≤5%) who were prescribed purified FIX concentrate, as determined by the treating physician. The planned observation period for each patient was either 12 months or ≥50 exposure days, whichever occurred first. The primary endpoints were the occurrence of treatment-related adverse events (AEs) and serious AEs (SAEs), and inhibitor development.

Results: Thirteen male patients (mean ± standard deviation age, 3.80 ± 1.76 years) enrolled and received ≥1 treatment with purified FIX concentrate. Thirty-two AEs were reported in 6 patients; 4 were SAEs. No AEs were considered related to purified FIX concentrate. No patients developed inhibitory antibodies. Inhibitor testing was not conducted in 2 patients. Eighteen bleeding episodes were treated with purified FIX concentrate in 6 patients. Hemostatic efficacy was rated as either "excellent" or "good" in all patients with an available rating.

Conclusion: No treatment-related AEs were reported, and purified FIX concentrate was shown to be effective in treating and preventing bleeding episodes in pediatric patients ≤6 years old with hemophilia B.

Introduction: Outcomes for patients with multiple myeloma has significantly improved through the years. This is mainly related to the use of novel agents.

Methods: This is a retrospective study that reviewed presentation and outcome of 139 patients with multiple myeloma at the Windsor Essex Regional Cancer Centre from Jan. 1, 2015 to Dec. 31, 2019. Median age was 71 years and most patients had higher risk disease (65.5% either R ISS stage II or III). 30% had high risk FISH for myeloma including del.17P, t (4:14), t (14:16) and Gain (1q21). In terms of presentation, 38.8% had anemia (hemoglobin <100g/L), 18.7% had hypercalcemia, 74.1% had skeletal lytic lesions, 38.8% had pathologic fracture and 17.3% had plasmacytoma.

Results: Almost all (92%) of the patients were treated using at least one novel agent (proteasome inhibitor or immunomodulators [ImiDs]). Cyclophosphamide, bortezomib, and dexamethasone (CyBorD) was the most used treatment regimen (48.9%) followed by bortezomib, melphalan and prednisone (BMP) at 28.8% and lenalidomide, dexamethasone (LenDex) at 14.4%. With respect to response to therapy, 51.8% had at least Very good partial response (VGPR), while 9.4% had progressive disease. 33% had autologous stem cell transplant. After a median follow up of 2.4 years, median overall survival was 3.7 years. 2 years overall survival and relapse-free survival were 70% and 83%, respectively.

Discussion: Our study showed comparable outcome for patients with multiple myeloma despite older age and higher risk disease. Outcome is expected to improve with the introduction of more novel agents.

Background: Anemia is among the major public health problems that cause significant morbidity and mortality among children around the world. Anemia in children of age 6 months to 5 years is a major health problem in most developing world countries with estimated prevalence of about 43%.

Objective: To determine the magnitude, associated factors and morphological types of anemia among hospitalized 6-59 months age children from June 15 to October 15, 2022 at Jimma Medical Center, southwest Ethiopia.

Methodology: Hospital-based cross- sectional study design was conducted from June 15 to October 15, 2022 at Jimma Medical Center, involving 383 hospitalized children aged 6-59 months by employing convenient sampling technique. Data of sociodemographic characteristics and other associated factors of the study individuals waere collected using a pre-structured questionnaire. Clinical data were collected by physical examination and from history of client by medical interns and nurses. Then 3 mL venous blood was collected and analyzed for complete blood count. Data were coded, cleared and entered into EpiData version 4.6 and exported to SPSS version 25 for analysis. Bivariable and multivariable binary logistic regression was used to identify associated factors.

Results: The overall prevalence of anemia among hospitalized 6-59 months age children was 57.2%; out of them 30.82% were moderate. In the present study children with malaria infection, AOR = 1.15 (95% CI: 0.017, 0.781), Cchildren with severe malnutrition, AOR = 2.046 (95% CI: 0.306, 1.366), and children with low family income, AOR = 2.6 (95% CI 0.475, 0.894) were independent variables associated with anemia.

Conclusion and recommendation: Anemia among study participants is found to be a severe public health problem. Based on this finding, more intervention is needed with health education on nutrition and child feeding.

Purpose: This study aimed to determine the survival rates and treatment outcomes of patients with childhood Acute Lymphoblastic Leukemia (ALL) in a single-center study at Indonesia.

Patients and methods: Factors contributing to the relapse and survival of ALL in Bandung, Indonesia, were evaluated. Data were collected from the medical record and the Indonesian Pediatric Cancer Registry (IPCAR). Subsequently, univariate and multivariate analyses were evaluated using Cox proportional hazard regression and Kaplan Meier was used for survival analysis. An analytic observational study was conducted on newly diagnosed children aged 1-18 with ALL from January 2019 to December 2022.

Results: A total of 137 children were included in the analysis, 30 (21,9%) were dropped out during treatment and 60.5% died during the study period. Most of the deaths occurred after relapse in 32 (38.5%) with a high early relapse (70.5%), occurring mainly during the maintenance phase (42.4%). At the one-year mark, the observed overall survival (OS) rate was at 36%, while event-free survival (EFS) was lower, at 19%. Univariate Cox regression analysis showed that the leucocyte counts at diagnosis (p=0.005) and response to induction phase (p < 0.008) was associated with the death of ALL. Furthermore, a response to induction phase was significant [hazard ratio 4.67 (CI 95%: 1.64-13.29); p = 0.004] in the multivariate analysis.

Conclusion: In conclusion, this study underscored the persistent challenges of high treatment discontinuation rates and the occurrence of very early relapses in low- to middle-income countries (LMICs), which significantly impacted the OS of children diagnosed with ALL.

Background: Nutritional anemia is a significant public health concern worldwide, particularly affecting young adults and children in Saudi Arabia, where inadequate nutrition is considered a primary contributing factor. This study aims to (i) examine the levels of serum iron, folate, and vitamin B₁₂ in young adult students, with a focus on identifying any deficiencies and their association with anemia; (ii) explore the prevalence of mixed-deficiency anemia resulting from deficiencies in serum iron, folate, and vitamin B₁₂ (iii) explore how sociodemographic characteristics and dietary habits influence serum iron, folate, and vitamin B₁₂ levels.

Materials and methods: This cross-sectional study encompassed 158 young adult students at Jazan University, Saudi Arabia. Blood samples were collected following a comprehensive questionnaire addressing sociodemographic and health characteristics. These samples were analyzed for complete blood count, serum iron, folate, and vitamin B₁₂ levels.

Results: The findings of this study revealed a significant decrease in serum iron levels, with 70.6% of males and 88% in females exhibiting reduced level. Additionally, low levels of folate were observed in 4% of the study population, while deficiency in vitamin B₁₂ was found in 2.2% of the study population. However, the simultaneous presence of low serum iron levels along with deficiencies in folate or vitamin B₁₂ was not observed in the study participants.

Conclusion: The study indicates that there is a high incidence of low serum iron and ferritin levels among university students in Saudi Arabia, which poses a considerable public health concern. Conversely, the prevalence of folate and vitamin B₁₂ deficiencies among the students was comparatively low, and notably, there were no cases where these deficiencies were observed alongside iron deficiency.