Journal of Blood Disorders and Transfusion最新文献

{"title":"Role of Circulating Endothelial Progenitor Cells in Micro VascularDamage in Sickle Cell Patients","authors":"B. Osa-Andrews, S. Oppong, H. Asare-Anane, G. Kpentey, T. John, B. Gyan","doi":"10.4172/2155-9864.1000389","DOIUrl":"https://doi.org/10.4172/2155-9864.1000389","url":null,"abstract":"The vascular wall participates in the pathogenesis of sickle cell disease (SCD). Circulating Endothelial progenitor cells (cEPCs) also play a key role in the vascular pathology of SCD, including the painful crisis. In previous investigations, reduced levels of cEPCs were found in conditions in which vascular injury is implicated such as myocardial infarction. The aim of this research is to study the role of cEPCs in the vascular pathology of SCD. cEPCs were enumerated using flow cytometry with the Flow Activated Cell Sorting machine (FACS). To differentiate the various genotypes-SS, SC, AS, AC and AA, Hb Electrophoresis was employed. Results showed that the median % cEPCs (CD34+/VEGF-2+) was lower in patients with SCD [0.555(0.4, 0.765)] than in healthy controls [(1.08(0.87, 1.39) (p=0.001)]. Patients in crisis had a higher cEPCs (0.65+0.39) than those in steady state (0.59+0.28) (p=0.522). SS group recorded the highest mean GGT (73.66+73.35). Only total cholesterol demonstrated a positive correlation (r=0.378, p=0.00814) with cEPCs in subjects; a trend unseen in healthy controls. Patients’ WBC, Hb and Liver enzymes- ALT, GGT, ALP showed no correlation with cEPCs. In healthy controls though, WBCs showed an inverse correlation with cEPCs (r=-0.6293, p=0.0003). SCD is as much a disease of endothelial dysfunction as it is a hemoglobinopathy that triggers erythrocyte polymerization: cEPC is a surrogate bio-marker for vascular function in SCD patients. The results suggest that SCD patients have depleted cEPCs compared with healthy controls. Oxidative stress, Nitric oxide activity, loss of CD133 during homing could influence progenitor cell populations. Total cholesterol positively correlates with cEPCs. High total cholesterol could spell the onset of painful crisis. Liver enzymes are not related to cEPCs correlatively even though the liver is involved in endothelial injury in SCD patients. SS individuals tend to have a high GGT. Overall, we have shown the direct correlation between total cholesterol and cEPCs in sickle cell patients.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"82 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121893712","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":"Fatal Delayed Hemolytic Transfusion Reaction and Hyperhemolysis without Detectable Alloantibodies or Autoantibodies in a Patient with Sickle Cell Disease: A Case Report and Literature Review","authors":"Basile Nsimba, A. Habibi, P. Bartolucci, D. Tonduangu, C. Duvoux, N. Prost, M. Marc, Etti, Mekontso-Dessap, F. Galactéros, P. Morel","doi":"10.4172/2155-9864.1000388","DOIUrl":"https://doi.org/10.4172/2155-9864.1000388","url":null,"abstract":"Background: Sickle cell disease (SCD) is one of the most commonly inherited diseases worldwide and one of the most frequently occurring genetic disorders in France. Delayed hemolytic transfusion reaction (DHTR) is a classic complication in patients with SCD who undergo blood transfusions, and this condition may lead to hyperhemolysis syndrome (HS). DHTR is described as a transfusion complication and is often associated with anti-RBC antibodies. \u0000Case report: We report the death of a 47-year-old man of Martinican origin, with sickle cell disease (SCD) and no history of alloimmunization, who was admitted to the Sens Medical Center in July 2015 due to a vaso-occlusive crisis (VOC). An antibody screening test based on the gel technique was conducted and no anti-RBC antibodies were detected. The patient was readmitted with a VOC two days after discharge and subsequently developed DHTR/HS after transfusion episodes. He succumbed to acute complications involving severe multiple organ failure after being transferred to Henri-Mondor University Hospital in Creteil. This case report was made available thanks to our Haemovigilance Network. \u0000Conclusion: This case demonstrates the importance of DHTR prevention using national guidelines to carefully assess indications for RBC transfusion in patients with SCD. Early diagnosis is crucial to prevent this life-threatening complication. Special attention should be given to less well-known and less-well monitored patients who may be at high risk for DHTR. In any event, DHTR/HS without detectable anti-RBC antibodies presents a clinical and biological challenge to our understanding of this disorder.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"2 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116851334","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":"The Role of Factor V Leiden 1691G>A and Prothrombin Gene 20210G>A Mutations in Hypercoagulable State Associated with Venous Thromboembolism among Sudanese Patients","authors":"Alfatih Aboalbasher Yousif, A. M. Muddathir, E. Elamin, A. Alhadi","doi":"10.4172/2155-9864.1000386","DOIUrl":"https://doi.org/10.4172/2155-9864.1000386","url":null,"abstract":"Background: Factor V Leiden (FVL) 1691G>A and Prothrombin (PRT) 20210G>A mutations are the most frequent hereditary cause of venous thrombosis in Caucasian and less frequency in African. The aim of this study was to detect the frequency of FVL 1691G>A and PRT 20210G>A mutations among Sudanese venous thromboembolism (VTE) patients. Material and Methods: This was descriptive Cross sectional study in which a total of 176 Sudanese subjects were enrolled in the period between July 2015 and July 2016. Among them, 38 apparently healthy Sudanese individuals as controls and 138 patients (47 males and 91 females), age range 18-90 with documented VTE confirmed by Duplex Doppler ultrasound at Khartoum Teaching Hospital and Sudan Heart Institute were included. Result: In this study, the mean age was 48 years and 67% of total VTE patients were over the age of 40 years. Increased age was noted in the VTE patients with over 34% over the age of 60 years. The controlled subjects included significantly younger individual with 92.1% under the age of 50 and 81.5% under the age of 40 years. The FVL 1691G>A and PRT 20210G>A mutations were totally absent among the studied population. Conclusion: The FVL 1619G>A and PRT 20210G>A mutations were totally absent among Sudanese VTE patients.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"172 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126067015","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":"Saving a Life in Front of My Eyes","authors":"A. Qidwai, B. Siddiqui, N. Mansoor, S. Anwari, S. Punjwani","doi":"10.4172/2155-9864.1000385","DOIUrl":"https://doi.org/10.4172/2155-9864.1000385","url":null,"abstract":"Background: World Health Organization (WHO) assigned voluntary non-remunerated donation as the safest form of Blood donation. Moreover, it emphasized on developing strategies and program to induct regular donors in large quantity to maintain the highest possible quality of blood supply. However, the major bone of contention is the number of drop outs in blood donor pool, hence the blood donor agencies always keeps this thought process moving that how to affiliate a long-term relationship with blood donors. There is continuous search and effort for the development of a successful program which can strengthen the long term relation between the blood donor club and blood donor. Methods: It was therefore mandatory to put forward a novel idea which can bring a drastic change in blood supply campaigns and amalgamate the donors with the recipients through a humanitarian binding force. After an year of project development the concept of \"SALIFOME Saving A Life Infront Of My Eyes\" \" was coined by Dr Asim Qidwai which was then forwarded for rigorous pilot study and reviewed by a multidimensional team (MDT) comprising of Hematologist, Thalassemia experts, Blood Bank Experts, Nursing Educationist, Psychologist and Pharmacist. Result: SALIFOME wherever implemented would provide the following benefits to the health care system specifically systems associated with chronic blood disorders including but not limited to: donor immediate satisfaction, lateral communication, long term engagement of donors, learning experience for others, establishment of large blood donors pool, community education, cost effective solution, better utilization of human model. Conclusion: Our intention with SALIFOME was to start and initiate something which will provide the blood donor one strong motivational and sustain long term relationship among donors and donor club. This concept can be used as reference for other centers dealing with haemoglobinpathies.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"30 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133818795","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":"A Novel Missense Mutation of F 9 Gene in Hemophilia B Patients","authors":"L. K. Yuen, Z. Zakaria, Y. Yusoff, E. Esa, F. Afandi, Faraizah Abd Karim","doi":"10.4172/2155-9864.1000383","DOIUrl":"https://doi.org/10.4172/2155-9864.1000383","url":null,"abstract":"Background: Hemophilia B is an X-linked recessive disorder caused by mutations in the coding sequence of F9 gene, leading to dysfunctional Factor IX (FIX) protein. \u0000Objectives: This study is to identify novel and recurrent mutations in hemophilia B patients. \u0000Method and Materials: In this study, 9 hemophilia B patients were screened on 8 exons using polymerase chain reaction (PCR) and direct sequencing. \u0000Results: We identified 6 point mutations, including 4 missense mutations and 2 nonsense mutations. One of the six point mutations is a novel mutation (NM_000133.3:c.230T>G) which has not been reported previously in hemophilia B database. Single nucleotide transversion of Thymine to Guanine occurs at nucleotide position 230, leading to amino acids substitution from Valine to Glycine at codon 77 in Gla domain. This amino acid substitution affects the protein structure and function in the Gla domain of FIX protein. Seven prediction tools were shown highly consistent result in predicting this novel mutation. \u0000Conclusion: In this study, all point mutations were found in the coding sequence especially exon 2, exon 5 and exon 8 and distributed among Gla domain, EGF2 domain and SP domains. Novel mutation c.230T>G occurred at exon 2 of F9 gene which has damaging impact to decrease the stability of protein structure and dysfunction in Gla domain of FIX protein.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"37 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117121963","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":"Seroprevalence of HIV, HBV, HCV and Syphilis among Blood Donors in Western Maharashtra and a Newer Proposed Donor Screening Algorithm","authors":"Varsha G Sul, N. Deshpande, P. A. Gadgil","doi":"10.4172/2155-9864.1000384","DOIUrl":"https://doi.org/10.4172/2155-9864.1000384","url":null,"abstract":"Background: Blood is one of the important integral components of body constituents. The transfusion medicine has important role in saving life and also has great public health impact. \u0000Aim: This study highlights the seroprevalence of transfusion transmissible infections (TTI) as well as reviews the policies for blood collection from donors by modifying the algorithm of screening tests. \u0000Materials and methods: This is retrospective study where total of 33,783 blood units for a period of 3 years January 2012 to December 2014 were tested by ELISA methods approved by National AIDS Control organization (NACO) and RPR was carried out for screening of Syphilis. Results: The seroprevalence of HIV, HBsAg, HCV and syphilis was 0.9%, 3.2%, 0.35%, 0.04% respectively in total donors. \u0000Conclusion: The prevalence of transfusion transmissible infections is declining, but there is need to review the donor screening policies to improve the quality of transfusion medicine services.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"30 29","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"113954800","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":"Inferior Vena Cava Filters as a Potential Non-immune Cause of Low Serum Haptoglobin","authors":"L. Cooling, D. Giacherio, M. Elkins","doi":"10.4172/2155-9864.1000382","DOIUrl":"https://doi.org/10.4172/2155-9864.1000382","url":null,"abstract":"Objective: Serum haptoglobin level is commonly used as a marker of hemolysis. We hypothesized that inferior vena cava (IVC) filters may be a potential non-immune etiology for subclinical hemolysis and low haptoglobin in some patients, after encountering a patient with an IVC filter, mild macrocytic anemia and unexplained severe haptoglobinemia. IVC filters can be associated with pressure gradients and turbulent blood flow in the presence of trapped clots and thrombus formation. \u0000Methods: Prospective study in patients undergoing IVC filter placement at our institution over a 3-month period in 2008. All patients had a haptoglobin level prior to filter placement, and then daily for a period of 3-14 days. The absolute and relative change in haptoglobin compared to pre-procedure levels was determined. Results were evaluated by t-test (paired, 2-tail). Statistics and graphics were performed with commercial software. \u0000Results: A total of 22 patients underwent IVC filter placement. Four patients were excluded due to low preprocedure haptoglobin levels. At least one post-procedure haptoglobin values was available until day 3 in 18/18 eligible patients. Nine patients were followed for a period of 1-2 weeks. Although the majority of patients had an increase in haptoglobin after IVC filter placement, 3/9 (30%) had >50% decrease in haptoglobin by day 7, including one patient with severe haptoglobinemia by day 14. Patients with decreased haptoglobin levels also had decreases in platelet count over the same period. \u0000Conclusion: These results suggest that IVC filters may be another non-immune, device-related cause of decreased haptoglobin in some patients.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"25 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130154690","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":"Autologus Stem Cell Transplantation for Cancers: India: 2017","authors":"S. Raut","doi":"10.4172/2155-9864.1000379","DOIUrl":"https://doi.org/10.4172/2155-9864.1000379","url":null,"abstract":"India is a well-known destination for health tourism including hematopoietic stem cell transplantation for various cancers like multiple myeloma, Hodgkin and non-hodgkin lymphoma. Hematopoietic stem cell transplant is cheaper in India as compared to developed countries and there is a huge cost difference. Here, the numbers of stem cell transplant centres and transplant activity are increasing. In this paper, we summarise present status of autologus stem cell transplant for various cancers in India including the basics of autologus stem cell transplant procedure and reported statistics. This will add to scarcely reported literature in this topic as well as encourage the Indian stem cell transplant activity.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"68 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134408714","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":"Thrombotic Thrombocytopenic Purpura Refractory to Plasmapheresis Treated Successfully with Vincristine: A Case Report","authors":"W. Elrewihby, S. Zaghloul, N. Saber, Emad Hasan, Adel Elattar","doi":"10.4172/2155-9864.1000380","DOIUrl":"https://doi.org/10.4172/2155-9864.1000380","url":null,"abstract":"Introduction \u0000TTP is a life-threatening clinicopathologic disorder involving multiple organ systems, which remains a management challenge to physicians. If not treated promptly, TTP typically follows a progressive deteriorating course with irreversible renal failure, progressive neurologic deterioration, cardiac ischemia, and death. The immediate administration of plasma exchange has been the essential and urgent treatment for patients with a clinical diagnosis of TTP The reported incidence of patients who do not respond to plasma exchange (PE) [refractory TTP] and require additional therapy varies between 10% and 42%. \u0000Case report \u0000We have described a 24 year female patient of TTP who remained refractory after 27 sessions of PE combined with corticosteroids. We added a slow infusion of 2mg Vincristine (VCR) and followed by another 2 dosed of 1 mg with 6 days between each dose resulting in successful clinical and laboratory improvement. \u0000Conclusion \u0000We conclude that, VCR is a safe, inexpensive, readily available and rapidly acting agent and should be used from the start together with PE.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"20 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123297412","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":"Prophylactic Danshen Injection Combined with Prostaglandin E1 and Low-dose Heparin may prevent Hepatic Veno-occlusive Disease after Hematopoietic Stem Cell Transplantation: A Single-center Study in China","authors":"Wang Xiaoning, Chunhong Sun, Caili Guo, P. He, Mei Zhang","doi":"10.4172/2155-9864.1000378","DOIUrl":"https://doi.org/10.4172/2155-9864.1000378","url":null,"abstract":"Objective: To investigate the effect of Danshen injection combined with prostaglandin E1 and low-dose heparinon prevention of hepatic veno-occlusive disease (HVOD) after hematopoietic stem cell transplantation (HSCT). Methods: A total of 126 patients undergoing HSCT between January 2008 and June 2015, received a combined prophylactic regimen of intravenous Danshen 20-30 ml/day, prostaglandin E 120-30 μg/d, and subcutaneous injection of low-dose heparin (100 U/kg/day) for prevention of HVOD. The incidence of HVOD and adverse events associated with this prophylactic regimen were observed. \u0000Results: Among 126 patients, 65 received autologous peripheral blood stem cell transplant, 34 received HLAidentical sibling HSCT, six received HLA mismatched siblingHSCT, seven received HLA-matched unrelated HSCT, and 14 received HLA-mismatched unrelated HSCT. No adverse reaction or coagulation disorder associated with this prophylactic regimen was observed. Only one case with acute myeloid leukemia (CR2) developed HVOD seven days after receiving myeloablative chemotherapy with oral busulfan and cyclophosphamide followed by HLAmismatched unrelated peripheral blood stem cell transplantation. This patient died of HVOD. \u0000Conclusion: Danshen injection combined with prostaglandin E1 and low-dose heparin is a safe and effective regimen for the prevention of HVOD after HSCT.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"93 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2017-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124560914","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}