BMC Hematology最新文献

{"title":"Hemostatic state augmented with platelet indices among Sudanese diabetic septic foot.","authors":"Bashir Abdrhman Bashir,&nbsp;Mohamed Salih Ali","doi":"10.1186/s12878-018-0104-5","DOIUrl":"https://doi.org/10.1186/s12878-018-0104-5","url":null,"abstract":"<p><strong>Background: </strong>Diabetes mellitus is a very rampant metabolic disorder, particularly type II. It has many complications such as the septic foot. Diabetic septic foot (DSF) patients are at high risk for coagulation abnormalities as well as surgical hazards. Owing to the potential sequelae of coagulation and vascular abnormalities, this work aimed at studying the hemostatic state and platelet indices in diabetes type II patients with septic foot.</p><p><strong>Methods: </strong>A case-control study was conducted during the period from July to September 2017 at Dr. Awaad medical center, Red Sea State, Sudan. 57 diabetic patients with septic foot, aged between 17 and 78 years along with 57 non-diabetic subjects as control were enrolled. Sociodemographic data were collected using a structured questionnaire. Venipuncture blood was taken with necessary safety measures. Diabetes profile, coagulation studies as well as platelet indices were estimated. Data was analyzed using SPSS version 24.0 for windows. Ethical approval was considered and written consent from each participant was obtained.</p><p><strong>Results: </strong>The mean age of diabetic patients with septic foot and healthy controls were 48.49 ± 15.8 and 32.77 ± 14.0, respectively. The duration of the diabetes onset was 10.43 ± 9.5 years. Plasma prothrombin time (PT) value (12.61 ± 2.6 vs 13.67 ± 1.5, <i>P</i> < 0.009) was found to be significantly shorter in DSF compared to control. Plasma activated partial thromboplastin time (APTT) value was significant in diabetic septic foot (32.64 ± 5.2 vs 28.49 ± 4.13, <i>P</i> < 0.000), and thrombin time (TT) did not changed in DSF. Mean platelet volume (MPV), platelet distribution width (PDW), and platelet large cell ratio (P-LCR) values were significantly decreased in DSF compared to control (<i>P</i> < 0.013, 0.034, and 0.020, respectively). PDW values were positively correlated with PT, APTT, and D-Dimer (DD) (<i>r</i> = 0.28/<i>p</i> < 0.003, <i>r</i> = 0.29/<i>p</i> < 0.029, and <i>r</i> = 0.32/<i>p</i> < 0.016, respectively). FVIII activity (121.86 ± 174.4 vs 98.66 ± 31.83, <i>P</i> < 0.951) was insignificant with DSF, as the DD was also insignificant (<i>P</i> < 0.081).</p><p><strong>Conclusion: </strong>Diabetes mellitus is associated with prothrombotic tendency. Hypercoagulable state in DSF is indicated by shortened PT finding. PDW is a manifesting evidence that proves the presence of more reactive and aggregable platelets in DSF patients.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"11"},"PeriodicalIF":0.0,"publicationDate":"2018-05-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-018-0104-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36109156","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}

{"title":"Blood factory: which stem cells?","authors":"Maria Teresa Esposito","doi":"10.1186/s12878-018-0105-4","DOIUrl":"10.1186/s12878-018-0105-4","url":null,"abstract":"<p><p>Blood transfusions are often essential for treatment of severe anaemia and pregnancy complications. The unavailability of blood is a medical concern, especially in developing countries. New sources of red blood cells (RBC) are under investigation. Several studies have attempted to produce functional RBC from CD34+ haematopoietic stem cells (HSC) isolated from peripheral blood and umbilical cord blood, from embryonic stem cells (ESC) and induced pluripotent stem cells (iPSC). A recent article published in Nature Communications describes a novel model for generating RBC from a stable erythroid cell line obtained from bone marrow CD34+ haematopoietic stem cells (HSC). The cells generated by this method are phenotypically and functionally adult RBC, that resemble very well the donor RBC. In vivo experiments confirmed no difference in the survival of these RBC and donor RBC. The study therefore highlights that this immortalized line is a promising new source of adult RBC.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"10"},"PeriodicalIF":0.0,"publicationDate":"2018-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5946469/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36098905","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}

{"title":"Prevalence of thrombocytopenia before and after initiation of HAART among HIV infected patients at black lion specialized hospital, Addis Ababa, Ethiopia: a cross sectional study.","authors":"Gashaw Garedew Woldeamanuel,&nbsp;Diresibachew Haile Wondimu","doi":"10.1186/s12878-018-0103-6","DOIUrl":"https://doi.org/10.1186/s12878-018-0103-6","url":null,"abstract":"<p><strong>Background: </strong>Hematological abnormalities are common in HIV positive patients. Of these, thrombocytopenia is a known complication which has been associated with a variety of bleeding disorders. However, its magnitude and related factors have not been well-characterized in the era of highly active antiretroviral therapy (HAART) in Ethiopia. Therefore, this study aimed to assess the prevalence of thrombocytopenia before and after initiation of HAART among HIV positive patients attending Black Lion Specialized Hospital, Addis Ababa, Ethiopia.</p><p><strong>Methods: </strong>A cross sectional study was conducted from February to April 2017 in Black Lion Specialized Hospital, Addis Ababa, Ethiopia. A total of 176 patients on HAART were selected using simple random sampling techniques. Socio-demographic and clinical characteristics of the study patients were collected using structured questionnaire. Measurements of platelet counts and CD4 + T cell counts were made using Sysmex XT 2000i hematology analyzer and BD FACS Count CD4 analyzer, respectively. Statistical analysis of the data (Paired T- test and binary logistic regression) was done using SPSS version 20. <i>P</i>-value < 0.05 was considered as statistically significant.</p><p><strong>Results: </strong>A total of 176 patients (Age > 18 years old) were enrolled in this study and had a mean age of 40.08 ± 9.38 years. There was significant increase in the mean values of platelet counts (218.44 ± 106.6 × 10³/μl vs 273.65 ± 83.8 × 10³/μl, <i>p</i> < 0.001) after six months of HAART initiation compared to the baseline. Prevalence of thrombocytopenia before and after HAART initiation was 25 and 5.7% respectively. HIV patients whose CD4 counts < 200 Cells/μl were more likely to have thrombocytopenia than HIV patients whose CD4 count ≥350 Cells/μl. However, it was not statistically associated with prevalence of thrombocytopenia.</p><p><strong>Conclusions: </strong>This study has shown that the prevalence of thrombocytopenia after HAART initiation was decreased significantly. Based on our results, a number of study participants still had thrombocytopenia after initiation of HAART. Therefore, continuous screening for thrombocytopenia among HIV infected patients should be performed to decrease the risk of morbidity and mortality.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"9"},"PeriodicalIF":0.0,"publicationDate":"2018-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-018-0103-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36098904","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}

{"title":"Prevalence of cytopenias in both HAART and HAART naïve HIV infected adult patients in Ethiopia: a cross sectional study.","authors":"Tamirat Edie Fekene,&nbsp;Leja Hamza Juhar,&nbsp;Chernet Hailu Mengesha,&nbsp;Dawit Kibru Worku","doi":"10.1186/s12878-018-0102-7","DOIUrl":"https://doi.org/10.1186/s12878-018-0102-7","url":null,"abstract":"<p><strong>Background: </strong>In individuals infected with HIV, hematological abnormalities are common and are associated with increased risk of disease progression and death. However, the profile of hematological abnormalities in HIV infected adult patients is not known in Ethiopia. Thus, the aim of this study was to assess the hematological manifestations of HIV infection and to identify the factors associated with cytopenias in both HAART and HAART naïve HIV infected adult patients in Ethiopia.</p><p><strong>Method: </strong>We conducted a cross-sectional quantitative study of HIV-infected adult patients attending the ART follow-up clinic of Jimma University Specialized Hospital in Jimma, Ethiopia, from July 2012 to September 2012. We used a structured questionnaire to collect socio-demographic and clinical information. After interviewing, 4 ml of venous blood was drawn from each study subject for hematologic and immunologic parameters.</p><p><strong>Result: </strong>The prevalence of anemia, leucopenia, thrombocytopenia and lymphopenia among the study individuals were 51.5%, 13%, 11.1% and 5% respectively. Presence of opportunistic infection (<i>p</i> = 0.001), use of CPT (<i>p</i> = 0.04) and CD4 count < 200 cells/μl (<i>p</i> = 0.002) were associated with an increased risk of anemia.</p><p><strong>Conclusion: </strong>Hematologic abnormalities were common in HIV infected adult patients. Of the cytopenias anemia was the most common. Use of CPT was independently associated with increased risk of anemia and leucopenia. Therefore, large scale and longitudinal studies, giving emphasis on the association of CPT and cytopenia, are recommended to strengthen and explore the problem in depth.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"8"},"PeriodicalIF":0.0,"publicationDate":"2018-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-018-0102-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35989891","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}

{"title":"Prevalence of anemia before and after initiation of antiretroviral therapy among HIV infected patients at Black Lion Specialized Hospital, Addis Ababa, Ethiopia: a cross sectional study.","authors":"Gashaw Garedew Woldeamanuel,&nbsp;Diresibachew Haile Wondimu","doi":"10.1186/s12878-018-0099-y","DOIUrl":"https://doi.org/10.1186/s12878-018-0099-y","url":null,"abstract":"<p><strong>Background: </strong>Anemia is the most common hematological abnormality in Human immunodeficiency virus (HIV) positive patients and a significant predictor of its progression to AIDS or death. This study was aimed to assess the prevalence of anemia before and after initiation of antiretroviral therapy (ART) among HIV positive patients attending Black Lion Specialized Hospital, Addis Ababa, Ethiopia.</p><p><strong>Methods: </strong>A cross sectional study was conducted from January to April, 2017 in Black Lion Specialized Hospital, Addis Ababa, Ethiopia. A total of 255 patients on ART were selected using simple random sampling techniques. Socio-demographic and clinical characteristics of the study subjects were collected using structured questionnaire. Measurements of complete blood cell counts and CD4 + T cell counts were made using Sysmex XT 2000i hematology analyzer and BD FACS Count CD4 analyzer, respectively. Statistical analysis of the data (Chi-square, paired T-test, logistic regression) was done using SPSS version 20. A <i>p</i>-value < 0.05 was considered as significant.</p><p><strong>Results: </strong>Prevalence of anemia before and after ART initiation was 41.9 and 11.4% respectively. There are a significance differences in CD4 + T cell count, RBC count, hemoglobin values and RBC indices in HIV patients before and after ART initiation (<i>p</i>-value < 0.05). WHO clinical stages and CD4+ T cell counts were found to be associated with the prevalence of anemia before ART initiation. Among the total number of anemic cases, normocytic normochromic anemia was present in 71% of the cases before ART and in 58.6% of the cases after ART. The prevalence of macrocytic normochromic anemia before and after ART initiation was 4.7 and 27.6% respectively.</p><p><strong>Conclusions: </strong>It is evident from this study that there is a remarkable reduction in the prevalence of anemia after ART initiation. However, a significant proportion of HIV patients remained anemic after 6 months of ART initiation suggesting the need for routine screening and proper treatment of anemia to mitigate its adverse effects.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"7"},"PeriodicalIF":0.0,"publicationDate":"2018-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-018-0099-y","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35938767","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}

{"title":"A pilot study on the usefulness of peripheral blood flow cytometry for the diagnosis of lower risk myelodysplastic syndromes: the \"MDS thermometer\".","authors":"Ana Aires,&nbsp;Maria Dos Anjos Teixeira,&nbsp;Catarina Lau,&nbsp;Cláudia Moreira,&nbsp;Ana Spínola,&nbsp;Alexandra Mota,&nbsp;Inês Freitas,&nbsp;Jorge Coutinho,&nbsp;Margarida Lima","doi":"10.1186/s12878-018-0101-8","DOIUrl":"https://doi.org/10.1186/s12878-018-0101-8","url":null,"abstract":"<p><strong>Background: </strong>Immunophenotypic analysis of the bone marrow (BM) cells has proven to be helpful in the diagnosis of Myelodysplastic Syndromes (MDS). However, the usefulness of flow cytometry (FCM) for the detection of myelodysplasia in the peripheral blood (PB) still needs to be investigated. The aim of this pilot study was to evaluate the value of FCM-based PB neutrophil and monocyte immunophenotyping for the diagnosis of lower risk MDS (LR-MDS).</p><p><strong>Methods: </strong>We evaluated by 8-color FCM the expression of multiple cell surface molecules (CD10, CD11b, CD11c, CD13, CD14, CD15, CD16, CD34, CD45, CD56, CD64 and HLA-DR) in PB neutrophils and monocytes from a series of 14 adult LR-MDS patients versus 14 normal individuals.</p><p><strong>Results: </strong>Peripheral blood neutrophils from patients with LR-MDS frequently had low forward scatter (FSC) and side scatter (SSC) values and low levels of CD11b, CD11c, CD10, CD16, CD13 and CD45 expression, in that order, as compared to normal neutrophils. In addition, patients with LR-MDS commonly display a higher fraction of CD14⁺CD56⁺ and a lower fraction of CD14⁺CD16⁺ monocytes in the PB. Based on these results, we proposed an immunophenotyping score based on which PB samples from patients with LR-MDS could be distinguished from normal PB samples with a sensitivity 93% and a specificity of 100%. In addition, we used this score to construct the MDS Thermometer, a screening tool for detection and monitoring of MDS in the PB in clinical practice.</p><p><strong>Conclusions: </strong>Peripheral blood neutrophil and monocyte immunophenotyping provide useful information for the diagnosis of LR-MDS, as a complement to cytomorphology. If validated by subsequent studies in larger series of MDS patients and extended to non-MDS patients with cytopenias, our findings may improve the diagnostic assessment and avoid invasive procedures in selected groups of MDS patients.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"6"},"PeriodicalIF":0.0,"publicationDate":"2018-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-018-0101-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35935248","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}

{"title":"A rare case of avascular necrosis in sickle cell trait: a case report.","authors":"William J Sanders","doi":"10.1186/s12878-018-0098-z","DOIUrl":"https://doi.org/10.1186/s12878-018-0098-z","url":null,"abstract":"<p><strong>Background: </strong>Sickle cell trait is usually an asymptomatic presentation of a patient with slightly different hemoglobin molecule makeup than normal. It is similar to a more serious disease, sickle cell disease, in which a person's hemoglobin is mutated in such a way that causes their red blood cells to easily change shape in certain environmental and internal states; this causes red blood cells to adhere to the walls and occlude the lumen of the arteries in which they travel, leading to downstream effects secondary to ischemia. Sickle cell trait does not have these ischemic effects, usually.</p><p><strong>Case presentation: </strong>In this case, a young African American female patient presents to the clinic with severe right hip pain. Her past medical history includes sickle cell trait and asthma. She has not been symptomatic of her asthma for years and is not on therapy for it. The pain has lasted for several months and has not improved with anti-inflammatory medication. There is severe pain with internal and external rotation of the hip. The neurovascularity of the lower extremities is intact bilaterally. MRI of the femur shows stage 2 or 3 avascular necrosis of the femoral head, while X-rays of the femur are unremarkable. Non weight-bearing for several weeks was unsuccessful; shortly thereafter, the patient underwent core decompression of the right femoral head as well as starting bisphosphonates. The patient improved temporarily but regressed shortly thereafter. Her avascular necrosis worsened radiographically over the next several months. At this point, the only other option would be to do a total hip arthroplasty, but the patient may need several more throughout her lifetime due to the lifespan of the artificial replacement.</p><p><strong>Conclusion: </strong>There have only been scarce reports of avascular necrosis in patients with sickle cell trait. This manuscript presents such a case and includes the trials and tribulations associated with its management.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"5"},"PeriodicalIF":0.0,"publicationDate":"2018-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-018-0098-z","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35866572","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}

{"title":"More is less, less is more, or does it really matter? The curious case of impact of azacitidine administration schedules on outcomes in patients with myelodysplastic syndromes.","authors":"Rory M Shallis, Amer M Zeidan","doi":"10.1186/s12878-018-0095-2","DOIUrl":"10.1186/s12878-018-0095-2","url":null,"abstract":"<p><p>Myelodysplastic syndromes (MDS) encompass a diverse group of hematologic disorders characterized by ineffective and malignant hematopoiesis, peripheral cytopenias and significantly increased risk of progression to acute myeloid leukemia (AML). The hypomethylating agents (HMA) azacitidine and decitabine induce meaningful clinical responses in a significant subset of patients with MDS. Though never compared directly with decitabine, only azacitidine has improved overall survival (OS) compared to conventional care in a randomized trial in patients with higher-risk MDS. The azacitidine regimen used in this pivotal trial AZA-001 included administration at 75 mg/m²/day for 7 consecutive days in 28-day cycles (7-0 regimen). Given the logistical difficulties of weekend administration in the 7-0 regimen, as well as in efforts to improve response rates, alternative dosing schedules have been used. In a typical 28-day cycle, administration schedules of 3, 5, 10, and (with the oral version of azacitidine) 14 and 21 days have been used in clinical trials. Most trials that evaluated alternative administration schedules of azacitidine did so in lower-risk MDS and did not directly compare to the 7-0 schedule. Given the lack of randomized prospective studies comparing the 7-0 schedule to the other regimens of azacitidine in MDS, Shapiro et al. conducted a systematic review in an attempt to answer this question. Here we place the findings of this important work in clinical context and review the current knowledge and unresolved issues regarding the impact of administration schedules of azacitidine on outcomes of patients with both lower-risk and higher-risk MDS.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"4"},"PeriodicalIF":0.0,"publicationDate":"2018-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5796398/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35822976","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}

{"title":"Systematic review of azacitidine regimens in myelodysplastic syndrome and acute myeloid leukemia.","authors":"Roman M Shapiro,&nbsp;Alejandro Lazo-Langner","doi":"10.1186/s12878-017-0094-8","DOIUrl":"https://doi.org/10.1186/s12878-017-0094-8","url":null,"abstract":"<p><strong>Background: </strong>5-Azacitidine administered as a 7-day dosing regimen (7-0-0) is approved in high risk IPSS myelodysplastic syndrome (MDS) patients. Alternative regimens such as a 5-day (5-0-0) or 7-day with a weekend break (5-2-2) are commonly used. No randomized controlled trial has been done directly comparing all three dosing regimens. The objective of this study was to compare the efficacies of the 5-0-0, 5-2-2, and 7-0-0 regimens in MDS and AML.</p><p><strong>Methods: </strong>A systematic review was conducted using MEDLINE, EMBASE and CENTRAL. Eligible studies were randomized controlled trials (RCTs), observational prospective and retrospective studies. The primary clinical outcomes were Objective Response Rate (ORR) defined as the sum of complete response (CR), partial response (PR), and hematological improvement (HI) as defined by the IWG 2006 criteria. A meta-analysis of simple proportions was conducted using a random effects model with weights defined according to Laird and Mosteller. Comparisons between groups were not attempted due to the heterogeneity of study designs.</p><p><strong>Results: </strong>The only RCT directly comparing alternative azacitidine regimens showed no difference in ORR between the 5-0-0 and 5-2-2 regimens. All other RCTs compared a dosing regimen to conventional care. The pooled proportion of ORR was 44.8% with 95% CI (42.8%, 45.5%) for 7-0-0, 41.2% with 95% CI (39.2%, 41.9%) for 5-0-0, and 45.8% with 95% CI (42.6%, 46.4%) for 5-2-2.</p><p><strong>Conclusions: </strong>Indirect comparison of alternative azacitidine dosing regimens in MDS and AML shows a benefit for the 7-day regimen in attaining ORR. Additional RCTs are required to definitively address this comparison.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"3"},"PeriodicalIF":0.0,"publicationDate":"2018-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-017-0094-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35822975","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}

{"title":"Factor V Leiden G1691A and prothrombin G20210A mutations among Palestinian patients with sickle cell disease.","authors":"Fekri Samarah,&nbsp;Mahmoud A Srour","doi":"10.1186/s12878-018-0097-0","DOIUrl":"https://doi.org/10.1186/s12878-018-0097-0","url":null,"abstract":"<p><strong>Background: </strong>Vascular thrombosis is an important pathophysiological aspect of sickle cell disease (SCD). This study aimed to investigate the prevalence and clinical impact of factor V Leiden G1691A (FVL) and prothrombin G20210A mutations among Palestinian sickle cell disease (SCD) patients.</p><p><strong>Methods: </strong>A total of 117 SCD patients, including 59 patients with sickle cell anemia (SS), 33 patients with sickle β-thalassemia and 25 individuals with sickle cell trait (AS) were studied. The control group consisted of 118 healthy individuals. FVL and prothrombin G20210A mutations were determined by RFLP PCR.</p><p><strong>Results: </strong>Analysis of the clinical history of SCD patients revealed that seven patients have had vascular complications such as ischemic stroke or deep vein thrombosis. In SCD patients, the inheritance of the FVL mutation showed a significantly higher incidence of pain in joints, chest and abdomen as well as regular dependence on blood transfusion compared to SCD with the wild type. Age- and sex-adjusted logistic regression analysis revealed a significant association between FVL and sickle cell anemia with an odds ratio (OR) of 5.6 (95% confidence intervals [CI] of 1.91-39.4, <i>P</i> = 0.039) in SS patients. However, increased prevalence of the FVL in AS subjects and sickle β-thalassemia patients was not statistically significant compared to controls (OR 3.97, 95% CI 0.51-28.6, <i>P</i> = 0.17 and OR 3.59, 95% CI 0.35-41.6, <i>P</i> = 0.26, respectively). The distribution of prothrombin G20210A mutation among SCD patients compared to controls was not significantly different, thus our findings do not support an association of this mutation with SCD.</p><p><strong>Conclusions: </strong>FVL was more prevalent among SS patients compared to controls and it was associated with higher incidence of disease complications among SCD patients.</p>","PeriodicalId":37740,"journal":{"name":"BMC Hematology","volume":"18 ","pages":"1"},"PeriodicalIF":0.0,"publicationDate":"2018-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s12878-018-0097-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35768806","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}