Background: Transfusion of granulocytes obtained by apheresis is beneficial in febrile neutropenia (FN) but expensive and time-consuming. Buffy-coat-derived granulocytes could be an alternative. We studied the efficacy and safety of the administration of irradiated buffy-coat-derived granulocytes along with the standard of care in pediatric high-risk (HR) FN.
Methods: Sixty children ≤18 years with malignancy and chemotherapy-induced HR FN were randomized to either the granulocyte transfusion (GT) arm which received irradiated buffy-coat derived granulocyte transfusion along with the standard treatment or the standard treatment (ST) arm.
Results: Baseline characteristics, day-to-defervescence, antibiotic duration, hospital stay, and mortality were comparable between the groups. A significant difference was seen in days to achieve absolute neutrophil count (ANC) >500/mm3 in the 2 groups: 4.5 days (3-6.5) in the GT arm v/s 8 days (4-11) in the ST arm (P=0.01).
Conclusion: Buffy-coat-derived granulocyte transfusion was safe and led to early hematological recovery but was not associated with survival benefits. Future studies with earlier initiation in the intended dose could be undertaken to generate more evidence.
{"title":"A randomized controlled trial to explore the safety and efficacy of irradiated buffy-coat granulocytes in pediatric patients with febrile neutropenia.","authors":"Mohanaraj Ramachandran, Aditya Kumar Gupta, Jagdish Prasad Meena, Ashish Datt Upadhyay, Poonam Coshic, Rakesh Lodha, Rachna Seth","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Transfusion of granulocytes obtained by apheresis is beneficial in febrile neutropenia (FN) but expensive and time-consuming. Buffy-coat-derived granulocytes could be an alternative. We studied the efficacy and safety of the administration of irradiated buffy-coat-derived granulocytes along with the standard of care in pediatric high-risk (HR) FN.</p><p><strong>Methods: </strong>Sixty children ≤18 years with malignancy and chemotherapy-induced HR FN were randomized to either the granulocyte transfusion (GT) arm which received irradiated buffy-coat derived granulocyte transfusion along with the standard treatment or the standard treatment (ST) arm.</p><p><strong>Results: </strong>Baseline characteristics, day-to-defervescence, antibiotic duration, hospital stay, and mortality were comparable between the groups. A significant difference was seen in days to achieve absolute neutrophil count (ANC) >500/mm<sup>3</sup> in the 2 groups: 4.5 days (3-6.5) in the GT arm v/s 8 days (4-11) in the ST arm (P=0.01).</p><p><strong>Conclusion: </strong>Buffy-coat-derived granulocyte transfusion was safe and led to early hematological recovery but was not associated with survival benefits. Future studies with earlier initiation in the intended dose could be undertaken to generate more evidence.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 5","pages":"152-161"},"PeriodicalIF":0.0,"publicationDate":"2023-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10658032/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138457198","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ghazi H Mogharbel, Ahmad S Badawi, Amal Yaseen Zaman, Mehrevan M Abd Elmoniem, Ibrahim M Abdel-Rahman, Mariam Eid Alenazi, Fareed Akbar Shah, Mohamed Abdelnaem Aly, Syed Nazar Imam, Nusaybah Eid Alenazi, Salah Mohamed El Sayed
Hematological disorders are common medical ailments constituting an important cause of morbidity and mortality worldwide, which may be managed efficiently using different prophetic medicine remedies as adjuvants to current therapeutics. Prophetic medicine includes the body of knowledge about medicine that has been derived from the deeds, customs (sunnah), ahadith (sayings), actions, and agreements of Prophet Muhammad, peace be upon him. This review article aims at exploring the magnitude of therapeutic benefits of prophetic medicine remedies as adjuvant treatments to many different types of hematological disorders. Herein, we reviewed many published research studies throughout the literature to delineate the potential therapeutic benefits of prophetic remedies on hematological disorders. Several types of hematological disorders may benefit from prophetic medicine remedies that are rich in natural antioxidants that combat oxidative stress-induced harm e.g. nigella sativa, oral honey, camel milk and urine, Ajwa date fruits, olive oil, Zamzam water and figs. Many prophetic medicine remedies were reported to decrease the hematological cytotoxicity effects induced by different chemicals and are beneficial in treating anemias e.g. iron deficiency anemia, sickle cell anemia, thalassemia, coagulopathies and hematological malignancies as leukemia and myeloma. These remedies treat or alleviate the different hematological disorders using different mechanisms e.g. modulating the immune function, treating deficiencies of different substances, protecting against toxins-induced cytotoxicity, decreasing platelets aggregation, suppressing clotting factors activation, exerting antineoplastic effects (enhancing cancer cells cytotoxicity) and inhibiting angiogenesis. Prophetic medicine remedies exert clinically significant therapeutic benefits for treating COVID-19 pandemic, anemia, thrombosis, thalassemia and blood cancers without inducing toxicity or side effects.
{"title":"Therapeutic benefits of prophetic medicine remedies in treating hematological diseases (A review article).","authors":"Ghazi H Mogharbel, Ahmad S Badawi, Amal Yaseen Zaman, Mehrevan M Abd Elmoniem, Ibrahim M Abdel-Rahman, Mariam Eid Alenazi, Fareed Akbar Shah, Mohamed Abdelnaem Aly, Syed Nazar Imam, Nusaybah Eid Alenazi, Salah Mohamed El Sayed","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Hematological disorders are common medical ailments constituting an important cause of morbidity and mortality worldwide, which may be managed efficiently using different prophetic medicine remedies as adjuvants to current therapeutics. Prophetic medicine includes the body of knowledge about medicine that has been derived from the deeds, customs (sunnah), ahadith (sayings), actions, and agreements of Prophet Muhammad, peace be upon him. This review article aims at exploring the magnitude of therapeutic benefits of prophetic medicine remedies as adjuvant treatments to many different types of hematological disorders. Herein, we reviewed many published research studies throughout the literature to delineate the potential therapeutic benefits of prophetic remedies on hematological disorders. Several types of hematological disorders may benefit from prophetic medicine remedies that are rich in natural antioxidants that combat oxidative stress-induced harm e.g. nigella sativa, oral honey, camel milk and urine, Ajwa date fruits, olive oil, Zamzam water and figs. Many prophetic medicine remedies were reported to decrease the hematological cytotoxicity effects induced by different chemicals and are beneficial in treating anemias e.g. iron deficiency anemia, sickle cell anemia, thalassemia, coagulopathies and hematological malignancies as leukemia and myeloma. These remedies treat or alleviate the different hematological disorders using different mechanisms e.g. modulating the immune function, treating deficiencies of different substances, protecting against toxins-induced cytotoxicity, decreasing platelets aggregation, suppressing clotting factors activation, exerting antineoplastic effects (enhancing cancer cells cytotoxicity) and inhibiting angiogenesis. Prophetic medicine remedies exert clinically significant therapeutic benefits for treating COVID-19 pandemic, anemia, thrombosis, thalassemia and blood cancers without inducing toxicity or side effects.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 4","pages":"130-142"},"PeriodicalIF":0.0,"publicationDate":"2023-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10509466/pdf/ajbr0013-0130.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41108436","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: The coronavirus disease 19 (COVID-19) infection has spread globally and caused a substantial amount of mortality and morbidity. Early detection of severe infections will improve care and reduce deaths. The use of hematological parameters in predicting COVID-19 disease severity, patient outcomes, and early risk stratification is limited. Therefore, the study was aimed at determining hematological parameters and their predictive value for assessing disease severity in laboratory-confirmed COVID-19 patients in Northwest Ethiopia.
Methods: A retrospective cross-sectional study was conducted at the University of Gondar comprehensive specialized hospital and Tibebe Ghion comprehensive specialized referral hospital on 253 patients diagnosed with COVID-19 and admitted between March 2021 and February 2022. Data were extracted, and entered into Epi-data 4.2.0.0, and analyzed using SPSS version 25 software. Hematological parameters were provided as the median and interquartile range (IQR). Categorical variables were represented by their frequency, and the χ2 test was applied to compare observed results with expected results. The receiver-operating curve (ROC) was used to establish the predictive value of hematological parameters for COVID-19 severity. A p-value < 0.05 was considered statistically significant.
Results: On a total of 253 patients, there were 43.87% severe cases, with a mortality rate of 26.9%. The ROC analysis showed the optimal cutoff values for hematological parameters were ANC (3370), lymphocyte (680), NLR (9.34), PLR (290.77), platelets (332,000), and WBCs (4390.65). The area under the curve (AUC) values for NLR (0.679) and ANC (0.631) were high, with the highest sensitivity and specificity, and could potentially be used to predict COVID-19 severity.
Conclusion: This study proved that high NLR and high ANC have prognostic value for assessing disease severity in COVID-19. Thus, assessing and considering these hematological parameters when triaging COVID-19 patients may prevent complications and improve the patient's outcome.
{"title":"Hematological parameters and their predictive value for assessing disease severity in laboratory-confirmed COVID-19 patients: a retrospective study.","authors":"Mezgebu Alemayehu Awoke, Ayinshet Adane, Belete Assefa, Solomon Getawa, Gebrehiwot Lema Legese, Mekonen Yimer","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>The coronavirus disease 19 (COVID-19) infection has spread globally and caused a substantial amount of mortality and morbidity. Early detection of severe infections will improve care and reduce deaths. The use of hematological parameters in predicting COVID-19 disease severity, patient outcomes, and early risk stratification is limited. Therefore, the study was aimed at determining hematological parameters and their predictive value for assessing disease severity in laboratory-confirmed COVID-19 patients in Northwest Ethiopia.</p><p><strong>Methods: </strong>A retrospective cross-sectional study was conducted at the University of Gondar comprehensive specialized hospital and Tibebe Ghion comprehensive specialized referral hospital on 253 patients diagnosed with COVID-19 and admitted between March 2021 and February 2022. Data were extracted, and entered into Epi-data 4.2.0.0, and analyzed using SPSS version 25 software. Hematological parameters were provided as the median and interquartile range (IQR). Categorical variables were represented by their frequency, and the χ<sup>2</sup> test was applied to compare observed results with expected results. The receiver-operating curve (ROC) was used to establish the predictive value of hematological parameters for COVID-19 severity. A <i>p</i>-value < 0.05 was considered statistically significant.</p><p><strong>Results: </strong>On a total of 253 patients, there were 43.87% severe cases, with a mortality rate of 26.9%. The ROC analysis showed the optimal cutoff values for hematological parameters were ANC (3370), lymphocyte (680), NLR (9.34), PLR (290.77), platelets (332,000), and WBCs (4390.65). The area under the curve (AUC) values for NLR (0.679) and ANC (0.631) were high, with the highest sensitivity and specificity, and could potentially be used to predict COVID-19 severity.</p><p><strong>Conclusion: </strong>This study proved that high NLR and high ANC have prognostic value for assessing disease severity in COVID-19. Thus, assessing and considering these hematological parameters when triaging COVID-19 patients may prevent complications and improve the patient's outcome.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 4","pages":"117-129"},"PeriodicalIF":0.0,"publicationDate":"2023-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10509465/pdf/ajbr0013-0117.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41097508","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Objectives: To assess the prevalence of coagulopathy in postoperative neurosurgical patients and correlate it with the outcome.
Materials and method: This longitudinal study was conducted in a tertiary care hospital in the Department of Pathology and Neurosurgery. Ethical approval was taken from the Institutional Ethical Committee - Human Research. Seventy-two (72) participants were recruited within 48 hours of surgery after obtaining consent. Complete clinical and surgical details were recorded. A 6.5 mL venous sample was collected and dispensed in two separate vials. The EDTA sample was run within 2 hours of collection on an automated hematology analyzer to obtain complete blood counts, including platelet count. The citrated sample was run on a fully automated coagulometer to determine PT, APTT, plasma fibrinogen, FVIII assay, and D-dimer levels. Subjects with a DIC-ISTH score of 5 or more were excluded. Coagulopathy was defined as three or more coagulation parameters deranged in a patient. All patients were followed up for the outcome. The outcome was correlated with coagulopathy, and a p-value less than 0.05 was considered statistically significant.
Results: The study found that the number of hemostatic parameters deranged correlated with outcome (P < 0.001). The proportion of patients with coagulopathy was 32/72 (44.4%), while those without coagulopathy were 40/72 (55.6%). Of patients with coagulopathy, 87.5% (28/32) had an adverse outcome, while 12.5% (4/32) had a favorable outcome. The difference was found to be statistically significant (P < 0.001).
Conclusions: Coagulopathy, defined as the derangement of three or more parameters, is a predictor of poor outcomes in postoperative neurosurgical patients. This timely recognition of coagulopathy can help triage patients requiring appropriate blood products, significantly reducing morbidity and mortality associated with postoperative neurosurgical patients.
{"title":"Coagulopathy in the absence of overt DIC in postoperative neurosurgical patients is a strong predictor of poor outcome.","authors":"Pooja Sharma, Richa Gupta, Satendra Sharma, Mrinalini Kotru, Gurubachan Singh, Meera Sikka","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Objectives: </strong>To assess the prevalence of coagulopathy in postoperative neurosurgical patients and correlate it with the outcome.</p><p><strong>Materials and method: </strong>This longitudinal study was conducted in a tertiary care hospital in the Department of Pathology and Neurosurgery. Ethical approval was taken from the Institutional Ethical Committee - Human Research. Seventy-two (72) participants were recruited within 48 hours of surgery after obtaining consent. Complete clinical and surgical details were recorded. A 6.5 mL venous sample was collected and dispensed in two separate vials. The EDTA sample was run within 2 hours of collection on an automated hematology analyzer to obtain complete blood counts, including platelet count. The citrated sample was run on a fully automated coagulometer to determine PT, APTT, plasma fibrinogen, FVIII assay, and D-dimer levels. Subjects with a DIC-ISTH score of 5 or more were excluded. Coagulopathy was defined as three or more coagulation parameters deranged in a patient. All patients were followed up for the outcome. The outcome was correlated with coagulopathy, and a <i>p</i>-value less than 0.05 was considered statistically significant.</p><p><strong>Results: </strong>The study found that the number of hemostatic parameters deranged correlated with outcome (P < 0.001). The proportion of patients with coagulopathy was 32/72 (44.4%), while those without coagulopathy were 40/72 (55.6%). Of patients with coagulopathy, 87.5% (28/32) had an adverse outcome, while 12.5% (4/32) had a favorable outcome. The difference was found to be statistically significant (P < 0.001).</p><p><strong>Conclusions: </strong>Coagulopathy, defined as the derangement of three or more parameters, is a predictor of poor outcomes in postoperative neurosurgical patients. This timely recognition of coagulopathy can help triage patients requiring appropriate blood products, significantly reducing morbidity and mortality associated with postoperative neurosurgical patients.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 4","pages":"110-116"},"PeriodicalIF":0.0,"publicationDate":"2023-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10509467/pdf/ajbr0013-0110.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41096125","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Loubna Abdel-Hadi, Yendry Ventura Carmenate, Yandy Marx Castillo-Aleman, Samira Sheikh, Aya Zakaria, John Phillips
Sickle Cell Disease (SCD) is one of the most inherited hematologic diseases affecting humans. Clinically, there is a progressive multiorgan failure and increased mortality in severe cases. The highest prevalence is in West Africa, India, the Mediterranean region, and Middle East countries. Hydroxyurea was the primary drug available for SCD and remains first-line therapy for patients with SCD. Three additional drug therapies, L-glutamine, Voxelotor, and Crizanlizumab, have been approved as adjunctive agents. However, none of these treatments are curative. Effective cell-based therapies are available, such as red blood cell (RBC) exchange and the only curative therapy is hematopoietic stem cell transplantation (HSCT). Gene-editing now shows promise in treating SCD and the β-thalassemias. Recent clinical trials have proven that this therapeutic strategy is effective, however costly. Despite the availability of safe and effective drug treatments, questions focusing on the overall value of these drugs exist in light of rising healthcare costs including hospitalizations and medical interventions. Herein, we report a cost-effective evaluation that can guide future efforts in making decisions towards HSCT as cell therapy treatment in SCD patients.
{"title":"Treatment of sickle cell disease - options and perspective.","authors":"Loubna Abdel-Hadi, Yendry Ventura Carmenate, Yandy Marx Castillo-Aleman, Samira Sheikh, Aya Zakaria, John Phillips","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Sickle Cell Disease (SCD) is one of the most inherited hematologic diseases affecting humans. Clinically, there is a progressive multiorgan failure and increased mortality in severe cases. The highest prevalence is in West Africa, India, the Mediterranean region, and Middle East countries. Hydroxyurea was the primary drug available for SCD and remains first-line therapy for patients with SCD. Three additional drug therapies, L-glutamine, Voxelotor, and Crizanlizumab, have been approved as adjunctive agents. However, none of these treatments are curative. Effective cell-based therapies are available, such as red blood cell (RBC) exchange and the only curative therapy is hematopoietic stem cell transplantation (HSCT). Gene-editing now shows promise in treating SCD and the β-thalassemias. Recent clinical trials have proven that this therapeutic strategy is effective, however costly. Despite the availability of safe and effective drug treatments, questions focusing on the overall value of these drugs exist in light of rising healthcare costs including hospitalizations and medical interventions. Herein, we report a cost-effective evaluation that can guide future efforts in making decisions towards HSCT as cell therapy treatment in SCD patients.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 2","pages":"61-70"},"PeriodicalIF":0.0,"publicationDate":"2023-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10195315/pdf/ajbr0013-0061.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9858420","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Acute myeloid leukemia with normal cytogenetics (CN-AML) is the largest group of AML patients with very heterogenous patient outcomes. The revised World Health Organization classification of the hematolymphoid tumours, 2022, has incorporated AML with Nucleophosphmin1 (NPM1) and CCAAT/enhancer binding protein-alpha (CEBPA) mutations as distinct entities. Despite the existing evidence of the prognostic relevance of FMS-like tyrosine kinase-3 internal tandem duplication (FLT3-ITD) in AML, it has not been included in the revised classification.
Method: In this prospective study, we determined the prevalence of NPM1, CEBPA, and FLT3 gene mutations in 151 de novo CN-AML adult patients (age ≥18 years) in a tertiary care hospital in north India. Additionally, the prognostic relevance of these mutations was also evaluated.
Results: NPM1, FLT3-ITD, and CEBPA mutations were found in 33.11%, 23.84%, and 15.77% of CN-AML patients, respectively. CEBPA mutations were found at 3 domains: transactivation domain 1 (TAD1) in 10 (6.62%), transactivation domain 2 (TAD2) in 5 (3.31%), and basic leucine zipper domain (bZIP) in 11 (7.82%) patients. Patients with NPM1 mutation had better clinical remission rate (CR) (P=0.003), event-free survival (P=0.0014), and overall survival (OS) (P=0.0017). However, FLT3-ITD and CEBPA mutations did not show any association with CR (P=0.404 and 0.92, respectively). Biallelic CEBPA mutations were found in 12 (7.95%) patients and were associated with better OS (P=0.043).
Conclusions: These findings indicate that NPM1 and CEBPA mutations can be precisely used for risk stratification in CN-AML patients.
{"title":"Prognostic relevance of <i>NPM1, CEBPA,</i> and <i>FLT3</i> mutations in cytogenetically normal adult AML patients.","authors":"Aparna Ningombam, Deepak Verma, Rajive Kumar, Jay Singh, M Shadab Ali, Avanish Kumar Pandey, Inder Singh, Sameer Bakhshi, Atul Sharma, Deepam Pushpam, Jayanth Kumar Palanichamy, Pranay Tanwar, Amar Ranjan Singh, Anita Chopra","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Acute myeloid leukemia with normal cytogenetics (CN-AML) is the largest group of AML patients with very heterogenous patient outcomes. The revised World Health Organization classification of the hematolymphoid tumours, 2022, has incorporated AML with Nucleophosphmin1 (<i>NPM1</i>) and CCAAT/enhancer binding protein-alpha (<i>CEBPA</i>) mutations as distinct entities. Despite the existing evidence of the prognostic relevance of FMS-like tyrosine kinase-3 internal tandem duplication (<i>FLT3-ITD</i>) in AML, it has not been included in the revised classification.</p><p><strong>Method: </strong>In this prospective study, we determined the prevalence of <i>NPM1, CEBPA,</i> and <i>FLT3</i> gene mutations in 151 de novo CN-AML adult patients (age ≥18 years) in a tertiary care hospital in north India. Additionally, the prognostic relevance of these mutations was also evaluated.</p><p><strong>Results: </strong><i>NPM1, FLT3-ITD,</i> and <i>CEBPA</i> mutations were found in 33.11%, 23.84%, and 15.77% of CN-AML patients, respectively. <i>CEBPA</i> mutations were found at 3 domains: transactivation domain 1 (TAD1) in 10 (6.62%), transactivation domain 2 (TAD2) in 5 (3.31%), and basic leucine zipper domain (bZIP) in 11 (7.82%) patients. Patients with <i>NPM1</i> mutation had better clinical remission rate (CR) (P=0.003), event-free survival (P=0.0014), and overall survival (OS) (P=0.0017). However, <i>FLT3-ITD</i> and <i>CEBPA mutations</i> did not show any association with CR (P=0.404 and 0.92, respectively). Biallelic <i>CEBPA</i> mutations were found in 12 (7.95%) patients and were associated with better OS (P=0.043).</p><p><strong>Conclusions: </strong>These findings indicate that <i>NPM1</i> and <i>CEBPA</i> mutations can be precisely used for risk stratification in CN-AML patients.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 1","pages":"28-43"},"PeriodicalIF":0.0,"publicationDate":"2023-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10017593/pdf/ajbr0013-0028.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9750771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The relation between the severity of COVID-19 and coexisting undiagnosed underlying thrombophilic conditions is not yet established. It may be possible that undiagnosed thrombophilia exaggerates an already pro-thrombotic state in COVID-19 patients and may be responsible for severe disease in absence of any known co-morbidity. The aim was to analyze the association of underlying thrombophilia with the severity of COVID-19 infection in post-COVID patients after a minimum of 6 weeks of recovery and to compare thrombophilia profile in severe versus non-severe COVID-19 patients. Forty severe and 40 non-severe COVID patients at least 6 weeks post recovery were selected for thrombophilia profile and complete blood count evaluation. The data were analyzed using Stata software, USA; version 13. The Chi-square test and Student's t-test were used to compare proportions and mean respectively. A total of 14/80 (17.5%) were positive for the thrombophilia screen. Protein C deficiency was noted in 6/40 (15%) severe COVID patients but not in the non-severe group. The Protein S deficiency was seen in 7/40 (17.5%) severe patients and only 1 patient was deficient in the non-severe group (2.5%). The mean Protein C and Protein S levels of severe and non-severe COVID patients were statistically significant (P-0.002) and (P-0.007) respectively. The difference in mean anti-COVID IgG antibody titer of severe and non-severe COVID patients was also statistically significant (P-0.0001). To Conclude, Protein C & S deficiencies were the commonest abnormalities detected in severe COVID patients. Positive thrombophilia profile and higher titers of anti-IgG COVID-19 antibodies were seen in a significant number of patients who had suffered from Severe COVID-19 than in non-severe infection, even after 6 weeks of recovery. Thereby, suggesting that underlying thrombophilia might have affected the severity of the disease.
{"title":"Inherited thrombophilia: undetected comorbidity complicating COVID-19 infection.","authors":"Divya Khatana, Poonam Rani, Swati Jain, Richa Gupta, Ashish Goel, Mrinalini Kotru","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The relation between the severity of COVID-19 and coexisting undiagnosed underlying thrombophilic conditions is not yet established. It may be possible that undiagnosed thrombophilia exaggerates an already pro-thrombotic state in COVID-19 patients and may be responsible for severe disease in absence of any known co-morbidity. The aim was to analyze the association of underlying thrombophilia with the severity of COVID-19 infection in post-COVID patients after a minimum of 6 weeks of recovery and to compare thrombophilia profile in severe versus non-severe COVID-19 patients. Forty severe and 40 non-severe COVID patients at least 6 weeks post recovery were selected for thrombophilia profile and complete blood count evaluation. The data were analyzed using Stata software, USA; version 13. The Chi-square test and Student's t-test were used to compare proportions and mean respectively. A total of 14/80 (17.5%) were positive for the thrombophilia screen. Protein C deficiency was noted in 6/40 (15%) severe COVID patients but not in the non-severe group. The Protein S deficiency was seen in 7/40 (17.5%) severe patients and only 1 patient was deficient in the non-severe group (2.5%). The mean Protein C and Protein S levels of severe and non-severe COVID patients were statistically significant (P-0.002) and (P-0.007) respectively. The difference in mean anti-COVID IgG antibody titer of severe and non-severe COVID patients was also statistically significant (P-0.0001). To Conclude, Protein C & S deficiencies were the commonest abnormalities detected in severe COVID patients. Positive thrombophilia profile and higher titers of anti-IgG COVID-19 antibodies were seen in a significant number of patients who had suffered from Severe COVID-19 than in non-severe infection, even after 6 weeks of recovery. Thereby, suggesting that underlying thrombophilia might have affected the severity of the disease.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 3","pages":"94-103"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10349295/pdf/ajbr0013-0094.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10182934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Acute myeloid leukemia (AML) is a heterogenous and challenging hematological malignancy with suboptimal outcomes. The implications of advanced technologies in the genetic characterization of AML have enhanced the understanding of individualized patient risk, which has also led to the development of new therapeutic strategies. A comprehensive study of novel mutations is essential to moderate the complicacies in patient management and achieve optimal outcomes in AML. In this review, we summarized the clinical relevance of important novel mutations, including TET2, ETV6, SATB1, EZH2, PTPN11, and U2AF1, which impact the prognosis of AML. TET2 mutation can lead to DNA hypermethylation, and gene fusion, and mutation in ETV6 disrupts hematopoietic transcription machinery, SATB1 downregulation aggravates the disease, and EZH2 mutation confers resistance to chemotherapy. PTPN11 mutation influences the RAS-MAPK signaling pathway, and U2AF1 alters the splicing of downstream mRNA. The systemic influence of these mutations has adverse consequences. Therefore, extensive research on novel mutations and their mechanism of action in the pathogenesis of AML is vital. This study lays out the perspective of expanding the apprehension about AML and novel drug targets. The combination of advanced genetic techniques, risk stratification, ongoing improvements, and innovations in treatment strategy will undoubtedly lead to improved survival outcomes in AML.
{"title":"Acute myeloid leukemia: novel mutations and their clinical implications.","authors":"Harshita Makkar, Ravi Kumar Majhi, Harsh Goel, Aditya Kumar Gupta, Anita Chopra, Pranay Tanwar, Rachna Seth","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Acute myeloid leukemia (AML) is a heterogenous and challenging hematological malignancy with suboptimal outcomes. The implications of advanced technologies in the genetic characterization of AML have enhanced the understanding of individualized patient risk, which has also led to the development of new therapeutic strategies. A comprehensive study of novel mutations is essential to moderate the complicacies in patient management and achieve optimal outcomes in AML. In this review, we summarized the clinical relevance of important novel mutations, including <i>TET2, ETV6, SATB1, EZH2, PTPN11,</i> and <i>U2AF1</i>, which impact the prognosis of AML. <i>TET2</i> mutation can lead to DNA hypermethylation, and gene fusion, and mutation in <i>ETV6</i> disrupts hematopoietic transcription machinery, <i>SATB1</i> downregulation aggravates the disease, and <i>EZH2</i> mutation confers resistance to chemotherapy. <i>PTPN11</i> mutation influences the RAS-MAPK signaling pathway, and <i>U2AF1</i> alters the splicing of downstream mRNA. The systemic influence of these mutations has adverse consequences. Therefore, extensive research on novel mutations and their mechanism of action in the pathogenesis of AML is vital. This study lays out the perspective of expanding the apprehension about AML and novel drug targets. The combination of advanced genetic techniques, risk stratification, ongoing improvements, and innovations in treatment strategy will undoubtedly lead to improved survival outcomes in AML.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 1","pages":"12-27"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10017594/pdf/ajbr0013-0012.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9499439","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hamed Mortazavi, Akbar Mohammadi, Elham Sharifian, Nima Firouzeh, Alex van Belkum, Hamed Ghasemzadeh-Moghaddam
Purpose: Additional knowledge on the epidemiology and recipients of blood transfusions will help health-care managers to estimate the future needs. The study was performed to define the blood transfusion rate based on gender, sex, and clinical features of patients receiving blood products in all hospitals of the North Khorasan province of Iran.
Methods: Data on blood transfusion implementation were extracted from blood bank documents. The data for all patients who received at least one blood product were collected from March 2018 to March 2019.
Results: Among blood transfused patients, the highest transfusion rate was for packed red blood cells (PRBC) (47.7%). The two other most frequently used products were fresh frizzed plasma (FFP) (27.2%) and platelets (PLT) (21.9%). The patients in the age group of 51-80 years received the majority of PRBCs and FFPs. Patients aged 21-40 and 61-70 yrs had the highest transfusion rates for PLT. Elderly female patients (57.4%) received more blood products than their male counterparts. The highest blood transfusion rates were among patients with neoplasms, anemia, gastrointestinal bleeding, and gastric diseases.
Conclusion: The primary Iranian blood recipients were elderly patients. Population aging is associated with an increase in the number of blood recipients and simultaneously declines the blood donors pool. It highlights the need for optimizing the use of blood in hospitals and having better strategies for overcoming the shortage of blood.
{"title":"The effect of aging on the epidemiology of blood transfusions in North Khorasan province, Iran.","authors":"Hamed Mortazavi, Akbar Mohammadi, Elham Sharifian, Nima Firouzeh, Alex van Belkum, Hamed Ghasemzadeh-Moghaddam","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Purpose: </strong>Additional knowledge on the epidemiology and recipients of blood transfusions will help health-care managers to estimate the future needs. The study was performed to define the blood transfusion rate based on gender, sex, and clinical features of patients receiving blood products in all hospitals of the North Khorasan province of Iran.</p><p><strong>Methods: </strong>Data on blood transfusion implementation were extracted from blood bank documents. The data for all patients who received at least one blood product were collected from March 2018 to March 2019.</p><p><strong>Results: </strong>Among blood transfused patients, the highest transfusion rate was for packed red blood cells (PRBC) (47.7%). The two other most frequently used products were fresh frizzed plasma (FFP) (27.2%) and platelets (PLT) (21.9%). The patients in the age group of 51-80 years received the majority of PRBCs and FFPs. Patients aged 21-40 and 61-70 yrs had the highest transfusion rates for PLT. Elderly female patients (57.4%) received more blood products than their male counterparts. The highest blood transfusion rates were among patients with neoplasms, anemia, gastrointestinal bleeding, and gastric diseases.</p><p><strong>Conclusion: </strong>The primary Iranian blood recipients were elderly patients. Population aging is associated with an increase in the number of blood recipients and simultaneously declines the blood donors pool. It highlights the need for optimizing the use of blood in hospitals and having better strategies for overcoming the shortage of blood.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 1","pages":"44-52"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10017592/pdf/ajbr0013-0044.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9499440","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Introduction: Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. Despite advancements in treatment, a significant proportion of children relapse. Recently, immunotherapy has gained momentum and is becoming popular, especially for relapsed and refractory cases. NK cells are an important part of tumor immunity and are involved in the direct killing of tumor cells. Their role in B-ALL has not been explored. Therefore, this study was conducted to correlate the number of NK cells with standard prognostic parameters in B-ALL.
Methods: 25 subjects with newly diagnosed B-ALL between 0-14 years were recruited for the study from Pediatric OPD or emergency of the hospital. Along with a complete hemogram and peripheral smear examination, immunophenotyping by flow cytometry was done at the time of diagnosis for NK cell enumeration. The number of NK cells was correlated with standard prognostic parameters using the spearman correlation coefficient.
Results: Baseline NK cell percentage demonstrated a significant negative correlation with Prednisone poor day 8 blast response (P value = 0.02, r value = -0.44) and positive MRD (P value = 0.01, r value = -0.49) at day 33. A negative correlation was also noticed between NK cell percentage and unfavorable cytogenetics (hypodiploidy), although it was not significant (P value = 0.06, r value = -0.38). The number of NK cells did not correlate with age, gender and WBC count. Therefore, evaluating NK cells at diagnosis may serve as a simple and useful parameter for prognostication and risk stratification.
Conclusion: It may be assumed that a higher percentage of NK cells is associated with improved outcomes and probably a better prognosis. NK numbers may serve as an early independent parameter predicting prognosis and survival in children with B-ALL, thus helping to decide individual therapeutic regimens.
{"title":"Higher baseline natural killer cell counts are associated with a lower 8-day blast count and lower day 33 minimal residual disease in children with pediatric B-acute lymphoblastic leukemia.","authors":"Nikita Sharma, Richa Gupta, Mrinalini Kotru, Sunil Gomber, Harsh Vardhan Gautam","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Introduction: </strong>Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. Despite advancements in treatment, a significant proportion of children relapse. Recently, immunotherapy has gained momentum and is becoming popular, especially for relapsed and refractory cases. NK cells are an important part of tumor immunity and are involved in the direct killing of tumor cells. Their role in B-ALL has not been explored. Therefore, this study was conducted to correlate the number of NK cells with standard prognostic parameters in B-ALL.</p><p><strong>Methods: </strong>25 subjects with newly diagnosed B-ALL between 0-14 years were recruited for the study from Pediatric OPD or emergency of the hospital. Along with a complete hemogram and peripheral smear examination, immunophenotyping by flow cytometry was done at the time of diagnosis for NK cell enumeration. The number of NK cells was correlated with standard prognostic parameters using the spearman correlation coefficient.</p><p><strong>Results: </strong>Baseline NK cell percentage demonstrated a significant negative correlation with Prednisone poor day 8 blast response (<i>P</i> value = 0.02, r value = -0.44) and positive MRD (<i>P</i> value = 0.01, r value = -0.49) at day 33. A negative correlation was also noticed between NK cell percentage and unfavorable cytogenetics (hypodiploidy), although it was not significant (<i>P</i> value = 0.06, r value = -0.38). The number of NK cells did not correlate with age, gender and WBC count. Therefore, evaluating NK cells at diagnosis may serve as a simple and useful parameter for prognostication and risk stratification.</p><p><strong>Conclusion: </strong>It may be assumed that a higher percentage of NK cells is associated with improved outcomes and probably a better prognosis. NK numbers may serve as an early independent parameter predicting prognosis and survival in children with B-ALL, thus helping to decide individual therapeutic regimens.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"13 1","pages":"53-60"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10017591/pdf/ajbr0013-0053.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9515043","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}