Background: Despite the confirmed favorable prognosis of childhood t(8;21) acute myeloid leukemia (AML), recent reports suggest heterogeneity in survival outcomes in this subtype of AML may be ...influenced by ethnicity. Therefore, we aimed to assess the outcome of childhood t(8;21) AML in an Arab population to evaluate if survival outcomes were inferior and determine the predictive relevance of additional cytogenetic abnormalities. Methods: This multicenter retrospective study analyzed 175 de novo AML children of 14 years of age or younger consecutively diagnosed between January 2005 and December 2012. Survival outcomes were analyzed and patients with t(8;21) were stratified on the basis of karyotype into sole and additional cytogenetic groups. Results: A total of 33 (18.9%) patients had t(8;21) AML. Complete remission (CR) was achieved in 31 (93.9%) patients. The 5-year overall survival, event-free survival, cumulative incidence of relapse (CIR), and remission death rates were 59.9 ± 9.2, 45.6 ± 9.1, 36.4, and 9.1%, respectively. Despite the administration of hematopoietic stem-cell-transplant salvage therapy in first relapse, five out of 11 (45.5%) relapsed patients died of disease. Subanalysis of sole vs. additional cytogenetic abnormalities revealed no significant difference in outcome. Conclusion: In the present study, childhood t(8;21) AML was associated with inferior survival and resistance to salvage therapy compared to reports from international groups. The inferior outcomes were unrelated to additional cytogenetic abnormalities. Further detailed genetic studies are warranted to unmask the biological and clinical differences between racial/ethnic groups. Given the high CR rate of childhood t(8;21) AML, further modification of postremission therapy to improve the CIR rate is needed.
Introduction: Sickle cell disease (SCD) is a genetic disorder that adversely impacts patients' physical and emotional well-being, yet research on the impact of SCD on quality of life is limited. The ...prevalence of SCD across Gulf Cooperation Council (GCC) countries ranges 0.24-5.8% (Abu-Shaheen et al. Expert Rev Hematol 2022). The Sickle Cell Health Awareness, Perspectives, and Experiences (SHAPE) survey aimed to broaden understanding of the global burden of SCD. This analysis reports the experiences of physicians in GCC countries treating patients with SCD who participated in the SHAPE survey within the context of insights obtained globally. Methods: Physicians from Brazil, Canada, France, Germany, the UK, the US, and 2 countries in the GCC (Saudi Arabia and the United Arab Emirates UAE) completed a 12-minute online survey comprising close-ended questions about their experiences of treating patients with SCD. Physicians practicing for 3-35 years and currently caring for ≥10 patients with SCD were eligible. Results:Of 219 physicians globally who responded, 30 (13.7%; Saudi Arabia and the UAE, n=15 each) were from GCC countries. Physicians in GCC countries on average were caring for 16 patients with SCD and had 16 years of experience treating patients with SCD. The most common symptoms of SCD reported to physicians in GCC countries were bone aches (95%) in patients aged ≤11 years, fatigue/tiredness and bone aches (92% each) in patients aged 12-17 years, and bone aches (100%) in patients aged ≥18 years. Bone aches and yellow eyes/skin/nails were symptoms reported by a significantly higher proportion of patients in all age groups to physicians in GCC countries than all surveyed physicians (Figure 1). Most physicians agreed early treatment of SCD can help with managing/preventing severe symptoms (80% in GCC countries vs 87% globally) and that treating hemolytic anemia can improve most aspects of SCD including, reducing end-organ damage (87% vs 84%). A significantly greater proportion of physicians in GCC countries, compared with all surveyed physicians, reported they were confident in their knowledge of the consequences of hemolytic anemia (93% vs 50%), and they were significantly more likely to attribute the development of SCD complications such as chronic anemia, kidney damage, and blood clots to hemolytic anemia (Figure 2). A significantly greater proportion of physicians in GCC countries, compared with all surveyed physicians, agreed end-organ damage is unavoidable (87% vs 59%), and they were reluctant to discuss the potential future challenges/complications of SCD with a patient or their family (57% vs 37%). Most physicians agreed they were optimistic about future treatments for SCD (73% in GCC countries vs 81% globally). A significantly greater proportion of physicians in GCC countries reported considering the cost/benefit ratio to the patient and/or health care system before recommending a treatment compared with all surveyed physicians (87% vs 64%). Physicians in GCC countries, compared with all surveyed physicians, reported a significantly smaller proportion of patients were treated with hydroxyurea (24% vs 43%) and opioids (10% vs 29%). The top concerns physicians in GCC countries had were their patients not understanding the long-term effects/progression of SCD (47%), only seeking treatment/care when experiencing crises (47%), and lack of community support for patients (40%). Physicians reported wanting more support to educate their patients about the long-term effects of SCD (93% in GCC countries vs 83% globally). Conclusions: Physicians in GCC countries identified the symptom(s) most frequently reported by patients aged ≤11 years and ≥18 years as bone aches, and by patients aged 12-17 years as bone aches and fatigue/tiredness. Most physicians in GCC countries agreed end-organ damage is unavoidable, but over half were reluctant to discuss the potential future complications of SCD with patients and their families. Findings from the SHAPE survey underline the need for treatments that target specific SCD symptoms and delay end-organ damage, as well as the need of physicians in GCC countries for greater support when treating patients with SCD.
During the coronavirus disease (COVID-19) pandemic, patients with cancer in rural settings and distant geographical areas will be affected the most by curfews. Virtual management (telemedicine) has ...been shown to reduce health costs and improve access to care.
The aim of this survey is to understand oncologists' awareness of and views on virtual management, challenges, and preferences, as well as their priorities regarding the prescribing of anticancer treatments during the COVID-19 pandemic.
We created a self-administrated electronic survey about the virtual management of patients with cancer during the COVID-19 pandemic. We evaluated its clinical sensibility and pilot tested the instrument. We surveyed practicing oncologists in Gulf and Arab countries using snowball sampling via emails and social media networks. Reminders were sent 1 and 2 weeks later using SurveyMonkey.
We received 222 responses from validated oncologists from April 2-22, 2020. An awareness of virtual clinics, virtual multidisciplinary teams, and virtual prescriptions was reported by 182 (82%), 175 (79%), and 166 (75%) respondents, respectively. Reported challenges associated with virtual management were the lack of physical exam (n=134, 60%), patients' awareness and access (n=131, 59%), the lack of physical attendance of patients (n=93, 42%), information technology (IT) support (n=82, 37%), and the safety of virtual management (n=78, 35%). Overall, 111 (50%) and 107 (48%) oncologists did not prefer the virtual prescription of chemotherapy and novel immunotherapy, respectively. However, 188 (85%), 165 (74%), and 127 (57%) oncologists preferred the virtual prescription of hormonal therapy, bone modifying agents, and targeted therapy, respectively. In total, 184 (83%), 183 (83%), and 176 (80%) oncologists preferred to continue neoadjuvant, adjuvant, and perioperative treatments, respectively. Overall, 118 (53%) respondents preferred to continue first-line palliative treatment, in contrast to 68 (30%) and 47 (21%) respondents indicating a preference to interrupt second- and third-line palliative treatment, respectively. For administration of virtual prescriptions, all respondents preferred the oral route and 118 (53%) preferred the subcutaneous route. In contrast, 193 (87%) did not prefer the intravenous route for virtual prescriptions. Overall, 102 (46%) oncologists responded that they would "definitely" prefer to manage patients with cancer virtually.
Oncologists have a high level of awareness of virtual management. Although their survey responses indicated that second- and third-line palliative treatments should be interrupted, they stated that neoadjuvant, adjuvant, perioperative, and first-line palliative treatments should continue. Our results confirm that oncologists' views on the priority of anticancer treatments are consistent with the evolving literature during the COVID-19 pandemic. Challenges to virtual management should be addressed to improve the care of patients with cancer.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Introduction: Sickle cell disease (SCD) is an inherited disorder that adversely impacts the physical and emotional well-being of patients and caregivers, yet research on the impact of SCD on quality ...of life (QoL) is limited. In a systematic review the prevalence of SCD in Gulf Cooperation Council (GCC) countries ranged 0.24-5.8% (Abu-Shaheen et al. Expert Rev Hematol 2022). The 3 countries with the highest prevalence were Bahrain (1.2-2.1%, 2 studies), Saudi Arabia (0.24-4.5%, 15 studies), and Oman (5.8%, 1 study). The Sickle Cell Health Awareness, Perspectives, and Experiences (SHAPE) survey aimed to improve our understanding of the global burden of SCD. This analysis reports the experiences of patients with SCD and caregivers in GCC countries who participated in the SHAPE survey within the context of insights obtained globally. Methods: The SHAPE survey included patients and caregivers from Brazil, Canada, France, Germany, the UK, the US, and 4 countries in the GCC (Bahrain, Oman patients only, Saudi Arabia, and the United Arab Emirates UAE). Participants completed a 12-minute face-to-face survey (GCC only) or online survey (other countries) comprising close-ended questions about their experiences with SCD. Patients with SCD aged ≥12 years and caregivers aged ≥18 years supporting a patient with SCD were eligible. Results: Of 919 patients and 207 caregivers globally who responded, 150 patients (16.3%; Saudi Arabia n=70, the UAE n=46, Bahrain n=19, and Oman n=15) and 50 caregivers (24.2%; Saudi Arabia n=25, the UAE n=17, and Bahrain n=8) were from GCC countries (Table). Symptoms experienced most frequently by patients in GCC countries and all surveyed patients, respectively, were fatigue/tiredness (91% vs 84%), bone aches (91% vs 69%), and vaso-occlusive crisis (VOC) pain (81% vs 71%). Compared with all surveyed patients, a significantly greater proportion of patients in GCC countries experienced fatigue/tiredness, bones aches, VOC pain, generalized pain, and signs of organ damage (Figure). Symptoms reported by a significantly smaller proportion of patients in GCC countries than by all surveyed patients were poor sleep/insomnia, poor appetite, memory/concentration issues, nausea, yellow eyes/nails/skin, vision difficulties/retinopathy, and leg ulcers. The top 3 symptoms patients in GCC countries reported as being most impactful in terms of the following categories were: (i) relationships/potential relationships (25% VOC pain, 19% low mood/feeling depressed, 16% bone aches); (ii) family (30% VOC pain, 12% bone aches, 11% low mood/feeling depressed); and (iii) long-term health prospects (29% bone aches, 23% VOC pain, 21% signs of organ damage). The average number of missed school/work days in the past month for patients was 3.9 in GCC countries and 7.5 globally. A smaller proportion of patients in GCC countries were optimistic about their future compared with all surveyed patients (35% vs 52%). The biggest concerns of caregivers of individuals with SCD were worsening of symptoms (54% in GCC countries vs 17% globally) and early loss of life (34% vs 39%). Approximately half of caregivers agreed that caring for a person with SCD had a big or noticeable impact on their family (52% in GCC countries vs 50% globally). The majority of caregivers agreed they were well-equipped to care for someone with SCD (80% in GCC countries vs 72% globally). A smaller proportion of caregivers in GCC countries were optimistic about the future of the person they care for with SCD compared with all surveyed caregivers (32% vs 62%). Conclusions: Findings from the SHAPE survey highlight the physical and emotional burden of SCD on patients and caregivers in GCC countries. Patients in GCC countries reported VOC pain, bone aches, and low mood/feeling depressed as symptoms that most affected their relationships/potential relationships and family. Caregivers in GCC countries expressed concerns about early loss of life and worsening SCD symptoms. Overall, these findings underline the need for effective treatments that alleviate symptoms and manage disease sequalae, and for additional resources to improve the QoL of patients and caregivers.
Background: No existing research has addressed respiratory tract infections (RTIs) in children with non-neutropenic fever (NNF) who are present during seasons of religious mass gatherings (Hajj, ...Ramadan), especially if this coincides with the influenza season. The treatment approach for RTI cases in NNF children during mass religious gatherings may not be applicable in the normal season.
Methods: This study includes a retrospective analysis of RTI in NNF children who were present during mass religious gathering seasons and/or flu season, and who were being treated for malignancy at Princess Noorah Oncology Center from May 1, 2016 through December 2017.
Results: A total of 4599 spikes in fever were identified in 124 non-neutropenic children in whom 455 spikes (9.9%, 455 out of 4599) were documented during mass religious gatherings and/or the flu season. NNF secondary to RTIs was documented in 59.7% of patients during the study seasons with a total of 245 fever spikes recorded. Ceftriaxone-based therapy was the main treatment approach for NNF including the RTI cases that were associated with 50% of subsequent hospitalization for further management. Among all the examined risk factors, a prolonged fever of more than three days was significantly associated with NNF secondary to RTI during mass religious gatherings if coincident with the flu season, OR=0.034 95% CI, 0.013 - 0.09 P-value <0.001.
Conclusions: The present study showed that the prevalence rate of NNF of RTI in NNF children increased during periods of mass gathering. There is need therefore for a national consensus to address NNF management during mass religious gatherings, particularly if coincident with the flu season.
Complex regional pain syndrome, characterized by pain, autonomic dysfunction, and decreased range of motion, developed after hepatitis B vaccination in four grade-6 children since the introduction of ...the vaccination program in British Columbia in1992. The reaction may result from injection trauma or may be secondary to a vaccine constituent.
The management of Refractory/Relapsed B-cell Acute Lymphoblastic Leukemia (R/R ALL) remains challenging. Incorporating blinatumomab in R/R ALL treatment has shown encouraging results. We describe the ...outcome and predictors of response in children receiving blinatumomab as a bridge to definitive therapy. Immunoglobulin (Ig) G and viral serology before and after therapy were evaluated. Thirty-three patients that failed standard first-line treatments due to relapsed ALL (n = 22), persistent minimal residual disease (MRD) (n = 8), or refractory disease (n = 3) received blinatumomab. Grade 2 toxicity occurred in 27.2% of patients. MRD remission (<0.01%) was achieved in 72.7% of patients. Pre-blinatumomab absolute lymphocyte count (ALC) and MRD/ALC ratio significantly associated with MRD-response. Patients with t(1;19) translocation had lower response rate, compared to all other cytogenetic categories (p = 0.013). One-year event-free survival (EFS) and overall survival (OS) were 69.2% and 79.7%, respectively. Analysis of OS and EFS showed pre-blinatumomab MRD level, ALC, MRD/ALC ratio, t(1;19), and post-blinatumomab MRD remission associated with survival. Following blinatumomab, 83% (15/18) of tested patients had low IgG levels. IgG seronegative status was observed in 83% (12/15) for varicella zoster, 35% (6/17) for herpes zoster, 18% (3/17) for cytomegalovirus, and 26% (5/17) for Epstein Barr virus. Blinatumomab produced encouraging results in children with R/R ALL and low disease burden bridging to definitive therapy. Incorporating baseline genetics and biomarkers may help identify subgroups likely to be responsive/resistant to therapy. Viral serological testing pre- and post-blinatumomab is recommended to optimize supportive and preemptive therapy.
Supplemental data for this article is available online at https://doi.org/10.1080/08880018.2022.2049936 .
Celotno besedilo
Dostopno za:
DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
PDF
