Austin Hematology最新文献

{"title":"Impact of Haplotypes on the Frequency of Morbid Complications in Homozygous SSFA2 Sickle Cell Disease in Cote D’ivoire","authors":"Silue Da, Ndhatz E, Ayemou R, K. B., Nanho Dc, Kamara I, Meite N, Bognini As, Botti Rp, Kouakou I, Djeket R, Koff Kg","doi":"10.26420/austinhematol.2021.1043","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1043","url":null,"abstract":"Background: Sickle cell disease is a constitutional hemoglobin disease witch poses a public health problem in Côte d’Ivoire due to its prevalence and complications. The homozygous form (SSFA2) is the most severe. The proportion of hemoglobin F by its property determines the haplotype. Authors wanted to determine the impact of these haplotypes on the frequency of morbid complications. Methods: Our study was a transversal type and analytical aims, occurred in the clinical hematology department of the University Hospital of Yopougon over a 3 months period. Our study included 150 SSFA2 patients with complications. The statistical test used was the student. Results: The mean age was 11 years, (6 months to 42 years). The sex ratio was 1.05. The mean rate of hemoglobin S was 86%, of which 17% had severe haplotype, 37% intermediate haplotype, and 45% benign haplotype. Infectious complications were the most frequent (58.72%) (Malaria 53.47%; bronchial pneumonia: 28.22%), followed by anemic complications (36.92%) and ischemic complications (4.36%). Deglobulization crisis was the major acute anemic complication (97.5%) followed by splenic sequestration (2.5%). Chronic anemic complications were dominated by leg ulcers (57.14%) followed by biliary lithiasis (42.86%). Aseptic necrosis of the femoral head was the most frequent ischemic complication (46.66%), followed by retinopathy (33.33%), and then stroke (20%). The severe haplotype was associated with a high frequency of complications in general and infectious complications in particular. (P=0.005) Conclusion: The clinical expression of the SSFA2 homozygous form and the occurrence of complications is closely related to the haplotype.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"37 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125440908","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Molecular Screening of Hemoglobin E Variant in Anemia Patients of Eastern UP Population, India","authors":"R. V, Y. U, K. P","doi":"10.26420/austinhematol.2021.1042","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1042","url":null,"abstract":"Hemoglobinopathy is one of the most common monogenic disorders. This is prevalent in South East Asia. More than 700 heboglobinopathies are reported worldwide, out of which HbS and HbE are also prevalent in India. The objective of the present study is to determine the frequency of HbE in Eastern UP population. After taking written consent, blood samples was collected from 350 individuals and genomic DNA was extracted from all the collected blood samples. PCR-RFP method was used to analyze the HbE mutation. Out of 350 samples analyzed, one individual was Heterozygous (HbE/N) and one individual was Homozygous (HbE/E) for HbE mutation. In conclusion, the βE allele frequency was observed as 0.42% in Eastern UP population. Percentage of both heterozygous and homozygous genotypes were 0.28%.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"61 24","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134224784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetics or Environmental Factor? Two Siblings with Myeloid Leukaemia: Case Report","authors":"Ibijola Aa, Adegbamigbe Oj, Okunlola Ai","doi":"10.26420/austinhematol.2021.1041","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1041","url":null,"abstract":"We present two female siblings of ages 28 and 21 years who developed myeloid Leukaemia concurrently within a year. No family history of haematological malignancy but there was positive history of undue exposure to herbicides and pesticides. Objective: To create more awareness on the need to observe bio safety measures when handling hazardous agents.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"26 6","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134426892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Successful Treatment of Pure Red Cell Aplasia and Chronic GVH with Rituximab after ABO Mismatched HSCT","authors":"S. Haidouri, Mehtat Em, S. Jennane, H. Elmaaroufi, M. Mikdame, K. Doghmi","doi":"10.26420/austinhematol.2021.1040","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1040","url":null,"abstract":"Background: Pure Red Cell Aplasia (PRCA) is a rare complication of ABO mismatched hematopoietic stem cell transplantation; there isn’t no standard of care, here we report a case of successful treatment by Rituximab in a refractory PRCA and chronic graft versus host disease. Case Presentation: A 26-year-old woman with PRCA following ABOmismatched allogeneic HSCT for chronic myeloid leukemia, associated with steroid refractory chronic hepatic graft versus host disease, treated with 4 doses of Rituximab 375mg/m² weekly, with an increase in her hemoglobin level and improvement of her liver’s enzymes. Conclusion: The interest of this case is to report the important therapeutic result of Rituximab, widely used in literature, especially if chronic Graft Versus host disease is associated.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"2015 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127755160","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immune Reconstitution Inflammatory Syndrome Associated with Hepatosplenic Candidiasis in a Patient with Acute Myeloblastic Leukemia: Possible Pathogenesis and Treatment in the Light of Current Knowledge","authors":"Z. Bıçakcı, D. Koca, G Bozbeyoglu","doi":"10.26420/austinhematol.2021.1039","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1039","url":null,"abstract":"Acquired immune deficiencies caused by different etiologies, promote invasive fungal infections. When this immunity begins to improve, it can induce an excessive inflammatory response defined as Immune Reconstitution Inflammatory Syndrome (IRIS). Hepatosplenic Candidiasis (HSC) can be considered a form of IRIS syndrome as it occurs following neutrophil recovery in patients treated for acute leukemia. Differentiating IRIS from a single fungal infection or treatment failure due to a similar clinical picture is a real diagnostic problem. Misdiagnosis and subsequently ineffective treatment with antifungal therapy instead of anti-inflammatory drugs, may lead fatal course of the disease. A deep and prolonged neutropenia developed after the first induction chemotherapy in our two and a half-year-old male patient who was followed up in our clinic with the diagnosis of Acute Myeloblastic Leukemia (AML). Our patient had fever, abdominal pain as well as his Gamma Glutamyl Transferase (GGT) and Alkaline Phosphatase (ALP) levels increased during neutropenia recovery. He was diagnosed with hepatosplenic candidiasis, by observing ‘target like abscesses’ on dynamic Magnetic Resonance Imaging (MRI) taken for his newly developing symptoms and laboratory findings while recovering neutropenia. After his first and third induction chemotherapy courses, his fever persisted although antifungal therapy, steroid treatment was initiated considering IRIS. After his re-intensification course, because of the same flare-up symptoms, we started immunglobulin in addition to steroid. With methylprednisolone and intravenous immunoglobulin, his symptoms improved and significant regression was observed in the lesions ‘target-like abscesses’ on MRI and in the laboratory values. Result: IRIS should be considered for patients with hepatic candidiasis whose have persistent fever despite appropriate antifungal therapy. Glucocorticoid should be started first for an anti-inflammatory effect.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"16 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121663365","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transfusion Related Acute Lung Injury (TRALI): A Rare Case after Single Packed Red Blood Cell (PRBC) Unit Transfusion","authors":"Balaban Pm","doi":"10.26420/austinhematol.2021.1038","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1038","url":null,"abstract":"Transfusion Related Acute Lung Injury (TRALI) is a rare but serious adverse event of allogeneic blood component transfusion, manifested typically by shortness of breath, a non-productive cough, fever, and hypotension, mostly seen after plasma component transfusion collected from female donors. We here present a rare case of TRALI requiring Intensive Care Unit (ICU) support after transfusion of single Packed Red Blood Cell (PRBC) unit collected from a male donor. The present case emphasizes that TRALI to be ruled out first in any patient showing acute /respiratory distress within 6hrs of transfusion, with prompt management and notification to transfusion services.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"18 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134240795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tricuspid Annular Plane Systolic Excursion as a Potential Marker of Hospital Utilization in Patients with Sickle Cell Disease","authors":"Seyller Hr, Harkins Ap, R. A, Mahrat S, Gutierrez D, C. J., Khong Ht","doi":"10.26420/austinhematol.2021.1037","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1037","url":null,"abstract":"Objective: Pulmonary Artery Hypertension (PAH) is an independent risk factor for increased morbidity in Sickle Cell Disease (SCD) patients. Tricuspid Annular Plane Systolic Excursion (TAPSE) can be used as a surrogate measure for PAH. The objective of this study was to determine whether lower TAPSE values are associated with increased Emergency Department (ED) and hospital utilization. Methods: TAPSE measurements were retrospectively obtained from bedside echocardiograms from a convenience sample of 28 SCD patients presenting to the ED with SCD pain. TAPSE was considered abnormal if <24.9mm, one standard deviation below the mean TAPSE for SCD patients. Medical records were reviewed to determine ED visits and hospital overnight stays over a two-year period. A t-test analysis and Pearson’s correlation were used for each variable. Results: The initial sample included 28 SCD patient encounters. TAPSE measurements were abnormal in 5 patients and normal in 23 patients. The mean number of ED visits/year for the abnormal and normal TAPSE group were 23.00 and 16.87, respectively (p=0.57) with moderate negative linearity (p=0.03). The mean number of hospitalized days for abnormal and normal TAPSE groups was 108.8 and 59.6, respectively (p=0.10) with moderate negative linearity (p=0.07). Conclusion: Lower TAPSE values (<24.9mm) in SCD patients were associated with higher ED and hospital utilization. If findings are replicated in larger studies, TAPSE may serve as a marker of morbidity in SCD patients presenting to the ED.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"44 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116899269","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retrospective Study of Blood Transfusion Complications in the Capital Region of Denmark from 1999-2017: Characteristics of Potentially “Dangerous” Blood Donors?s","authors":"Have Sb, Hother Ce, V. Jh, Dziegiel Mh, Hansen Mb, Ostrowski Sr","doi":"10.26420/austinhematol.2021.1036","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1036","url":null,"abstract":"Objectives: We hypothesized that the blood donors most frequently involved in complications would induce more and severe immunologic transfusion complications compared to other donors, i.e. potentially “dangerous”. Secondary aims were differences in demographic variables. Background: Donor-related mechanisms may contribute to allogeneic blood transfusion complications and may represent a dangerous treatment adverse event. Materials and Methods: By analyzing transfusion data from the Capital Region of Denmark from January 1, 1999 to December 31, 2017; 2,574,646 blood transfusions and 9,779 transfusion complications from 194,432 blood donors were included in our dataset. We divided donors into three groups based on the number of complications and complication frequency (potentially “dangerous” vs. two differently defined control groups i.e. control 1 and control 2), and compared the nature of transfusion complications and demographic variables by statistical analysis. Results: There were no differences in the proportion of complication types between the potentially “dangerous” donors and control donors, and no difference in the proportion of complications from RBCs, plasma or platelets according to ABO and RhD blood types. However, more potentially “dangerous” donors were female and had ABO blood type B compared to control donors (p<0.001 and p<0.01, respectively). The potentially “dangerous” donors were younger compared to control donors (40.36 years vs. 45.24 years and 42.84 years, p<0.001). Conclusion: The potentially “dangerous” did not display more/severe immunologic transfusion complications compared to control donors. However, they differed in regards to gender, age and blood type. Further research regarding the differences in complication frequency per donor and demographic variety is warranted.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"20 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131185706","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Artificial Intelligence Tools in the Global Deployment of Vaccines and Alternatives Modes of Immunotherapy for the Evolving SARS-CoV-2 Variants, From the UK Perspectivess","authors":"S. J","doi":"10.26420/austinhematol.2021.1034","DOIUrl":"https://doi.org/10.26420/austinhematol.2021.1034","url":null,"abstract":"This commentary is focused on a working title: “The story of a different year, with enormous challenges imposed by CoV-2 strains” and how we have responded to the way back to normality, where the use of newer artificial intelligence tools become extremely useful in the procedurals, patterns and big data analyses of the “new generation of vaccines and convalescent plasma therapy for management of CoV-2”. The goal is to highlights the current status of deployment of vaccines, from the UK perspectives, in two specific areas.","PeriodicalId":401162,"journal":{"name":"Austin Hematology","volume":"4 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127871179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}