Advances in Hematology最新文献

Obesity plays an essential role in the safety of pharmacologic drugs. There is paucity of data for direct oral anticoagulants (DOACs) in the obese, despite these agents becoming more widely used. The primary and secondary objectives of this study were to assess the safety and efficacy of DOACs in the overweight and obese populations when used for primary prophylaxis in the setting of non-valvular atrial fibrillation (NVAF) and for treatment of venous thromboembolisms (VTE). We conducted a retrospective cohort study in a large tertiary care center and obtained data through review of electronic health records. Among patients with NVAF and VTE on apixaban, there were no differences in rates of major bleeding (MB) and clinically relevant nonmajor bleeding (CRNMB) in the overweight and obese populations when compared to normal weight and underweight individuals. The multivariate adjusted analysis for rivaroxaban found that the odds of CRNMB for patients with BMI <25 was 5.37 (95% CI 1.50-19.32) times higher than that of BMI ≥25. Moreover, patients on medications that had known interactions with DOACs had 6.40 times higher odds of CRNMB than patients without such interactions (95% CI 1.49-27.57), which was not accounted for by the effects of aspirin and plavix alone. Efficacy was similar between all weight groups, for both apixaban and rivaroxaban. These results support previous analyses preformed in the large phase III trials and confirm that apixaban and rivaroxaban are safe in the overweight and obese.

This study aimed to determine the clinical impact of CD25⁺/CD123⁺ coexpression in adult B-cell acute lymphoblastic leukemia (B-ALL) cases. One hundred and twenty newly diagnosed B-ALL patients (≤60 years old) were included in this study. CD123 and CD25 expression on leukemic blast cells were assessed using flow cytometry. CD25⁺/CD123⁺ coexpression was detected in 40/120 B-ALL patients (33.3%). All B-ALL patients showed CD25⁺/CD123⁺ coexpression had lower induction of remission response and shorter overall survival as compared to B-ALL cases lacking coexpression. In conclusion, CD25⁺/CD123⁺ positive coexpression is a reliable flow cytometry marker for prediction of the outcome of adult B-ALL patients and could be used as a novel parameter for risk stratification of adult B-ALL cases.

Background: Imatinib mesylate is the gold standard for the treatment of all phases of Philadelphia-positive chronic myeloid leukemia. Patients on imatinib treatment may develop cytopenia due to drug toxicity. This study aimed to determine the types, grades, and time course of cytopenia in CML patients on imatinib at a Nairobi hospital.

Methods: This was a cross-sectional descriptive study of adult patients aged ≥18 years followed up at the Glivec International Patient Access Program (GIPAP) clinic from 2007 to 2015. Patients who developed cytopenia within 12 months of initiating imatinib were eligible. Clinical and hematologic data were retrieved from the patients' charts and entered into a study proforma. Measures of central tendency such as mean, median, mode, standard deviation, and variance were used for analysis.

Results: Sixty three percent (63.6%) of the 94 patients developed a monocytopenia, with anemia seen in 34%, neutropenia in 27.6%, and thrombocytopenia in 8% of the 94 patients. Anemia plus neutropenia was the most common bicytopenia at 12.7%. Pancytopenia was seen in only 5 of the 94 patients. Most of the cytopenia was grades 2 and 3. Anemia was present at baseline while neutropenia and thrombocytopenia developed within 12 months of imatinib initiation. Anemia resolved during the first 12 months of therapy while neutropenia and thrombocytopenia resolved within 24-36 months of treatment.

Conclusion: Monocytopenia, especially anemia, was the most common type of cytopenia. The cytopenia was predominantly grade 2, developed in majority of the patients within 6 months after imatinib initiation, and had resolved by 24-36 months after imatinib initiation.

Objectives: Evaluate the impact of a Quality and Safety Program (QSP) on the reduction of blood loss and transfusion needs in pediatric spinal deformity surgery, while defining risk factors for transfusion.

Background: Multimodal plan aiming to minimize transfusion needs has been shown to reduce transfusions and index rates in spinal deformity surgery. Anticipating blood loss and transfusion may help direct resources to patient needs or encourage reconsideration of the surgical plan.

Methods: This is a single-center retrospective study of prospectively collected data. Impact of this multimodal plan was studied on idiopathic deformities (Group A, 109 patients) and scoliosis associated with syndromic, neuromuscular, and muscular dystrophies (Group B, 100 patients), both before and after QSP.

Results: A decrease in total estimated blood loss was observed. In Group A, transfused patients decreased from 83.7% to 28% (p < 0.001, odds: 0.077), and, in Group B, from 98.7% to 66% (p < 0.01, odds: 0.038). Pearson's correlation identified patient body weight (r = 0.245, p=0.001) and Cobb angle (r = 0.175, p=0.017) as factors related to blood loss. A linear regression model to estimate hematic losses revealed that only body weight and transfusion showed predictive power, resulting in a low predictive model (R ² = 0.156; F(3,167) = 15.483, p < 0.001). A mediated model to explain blood loss was built based on a set of variables influencing transfusion which is, in turn, related to blood loss.

Conclusion: Transfusion needs in scoliosis surgery can be substantially reduced following a multimodal approach. The success of a program is strongly dependent on team effort, and the introduction of a risk assessment tool for transfusion needs indirectly assesses surgical risk, thus allowing relocation of resources to decrease blood loss.

This retrospective pilot study aimed to detect whether remaining dental/periodontal treatment need and periodontal inflammation after dental clearance would be associated with the initial therapy outcome of adult patients with acute leukemia undergoing induction chemotherapy. Different parameters were assessed from the patients' records: initial blood parameters, blood parameters during initial chemotherapy, leukemia/therapy related complaints, duration of fever, microbiological findings (blood and urine), as well as patients' survival. Dental treatment need was defined as the presence of at least one carious tooth; periodontal treatment need was determined by the presence of probing depth ≥3.5 mm in at least two sextants. To reflect periodontal inflammation, the periodontal inflamed surface area (PISA) was applied. Thirty-nine patients were included. A dental treatment need of 75% and periodontal treatment need of 76% as well as an average PISA of 153.18 ± 158.09 were found. Only two associations were detected: periodontal treatment need was associated with thrombocyte count after 7 days (p=0.03), and PISA was associated with erythrocyte count three days after induction of therapy (p=0.01). It can be concluded that remaining dental and periodontal treatment need as well as periodontal inflammation after dental clearance is not associated with the outcome of induction therapy in adult patients with acute leukemia.

Alpha-thalassemia is highly prevalent in the plural society of Brazil and is a public health problem. There is limited knowledge on its accurate frequency and distribution in the Amazon region. Knowing the frequency of thalassemia and the prevalence of responsible mutations is, therefore, an important step in the understanding and control program. Hematological and molecular data, in addition to serum iron and serum ferritin, from 989 unrelated first-time blood donors from Amazonas Hemotherapy and Hematology Foundation (FHEMOAM) were collected. In this study, the subjects were screened for -α ^3.7/4.2/^20.5, -^SEA, -^FIL, and -^MED deletions. Alpha-thalassemia screening was carried out between 2016 and 2017 among 714 (72.1%) male and 275 (27.9%) female donors. The aims of this analysis were to describe the distribution of various alpha-thalassemia alleles by gender, along with their genotypic interactions, and to illustrate the hematological changes associated with each phenotype. Amongst the patients, 5.35% (n = 53) were diagnosed with deletion -α ^-3.7 and only one donor with α ^-4.2 deletion. From the individuals with -α ^-3.7, 85.8% (n = 46) were heterozygous and 14.20% (n = 7) were homozygous. The frequency of the -α ^-3.7 deletion was higher in male (5.89%) than in female (4.0%). There is no significant difference in the distribution of -α ^-3.7 by gender (p = 0.217). The -α ^20.5, -^SEA, and -^MED deletions were not found. All subjects were analyzed for serum iron and serum ferritin, with 1.04% being iron deficient (n = 5) and none with very high levels of stored iron (>220 µg/dL). Alpha-thalassemia-2^3.7kb deletion was the most common allele detected in Manaus blood donors, which is a consistent result, once it is the most common type of α-thalassemia found throughout the world. As expected, the mean of hematological data was significantly lower in alpha-thalassemia carriers (p < 0.001), mainly homozygous genotype. Leukocytes and platelet count did not differ significantly. Due to the small number of individuals with iron deficiency found among blood donors, the differential diagnosis between the two types of anemia was not possible, even because minor changes were found among hematological parameters with iron deficiency and α-thalassemia. Despite this, the study showed the values of hematological parameters, especially MCV and MCH, are lower in donors with iron deficiency, especially when associated with α-thalassemia, and therefore, it may be useful to discriminate different types of microcytic anaemia. In conclusion, we believed screening for thalassemia trait should be included as part of a standard blood testing before blood donation. It should be noted that this was the f

Objectives: Essential thrombocythemia (ET) is one of the myeloproliferative neoplasms characterized by a sustained elevation of platelet numbers with a tendency for thrombosis and hemorrhage. The aim of this work is to establish the relation between calreticulin, factor V Leiden, prothrombin G20210A, and MTHFR mutations in ET patients and the thrombotic risk of these patients.

Methods: This study was carried out on 120 ET patients and 40 apparently healthy individuals as a control group.

Results: There were increases in WBCs, PLT counts, PT, fibrinogen concentration factor V Leiden, and MTHFR mutation in ET patients as compared to the control group (P < 0.05). Also, there were increases in WBCs, PLT counts, and hematocrit value in thrombosed ET patients as compared to the nonthrombosed ones (P < 0.05). On the contrary, there was no significantly statistical difference in ET patients with JAK2 V617F positive mutation versus the JAK2 negative group (P > 0.05) and in patients with cardiovascular risk factors versus patients with noncardiovascular risk factors (P > 0.05). ET patients with factor V Leiden, prothrombin gene, and CALR mutations were more prone to thrombosis (odds ratio 5.6, 5.7 and 4.7, respectively). On the contrary, JAk2V 617F and MTHFR mutations have no effect on the thrombotic state of those patients.

Conclusion: There is a significant increase risk of thrombosis in ET patients with CALR mutation, thrombophilic mutations, as well as factor V Leiden and prothrombin gene mutation with a risk of developing leukemic transformation.

Essential thrombocythemia is a rare hematological malignancy with good overall survival, but moderate to high risk of developing arterial or venous thrombosis lifelong. Different thrombotic risk scores for patients with essential thrombocythemia have been proposed, but only one of them (the IPSET-t scoring system) takes into account the classical cardiovascular risk factors as one of the scoring items. Currently, in clinical practice, the presence of cardiovascular risk factors in patients with diagnosis of ET rarely determines the decision to initiate cytoreductive therapies. In our study, we compared different risk models to estimate the thrombotic risk of 233 ET patients and the role of specific driver mutations and evaluated the impact that conventional cardiovascular risk factors (hypertension, cigarette smoking, diabetes, obesity, and dyslipidaemia) have on thrombotic risk in patients with ET. Perspective studies conducted on a polycentric large cohort of patients should be conducted to estimate the impact of cardiovascular risk factors in determining thrombosis in ET patients, evaluating the opportunity of initiating a cytoreductive therapy in patients with cardiovascular risk factors, even if classified into low to moderate risk groups according to other scoring systems.

Background: It is estimated that one out of every three Ghanaians has hemoglobin genotype mutation. This change in genetic make-up may result in genotypes such as HbAS, HbSS, and HbSC. Many children in low- and middle-income countries die even before they are diagnosed with sickle cell disease (SCD). In Africa, there are limited data on the incidence and prevalence of SCD and the Volta region of Ghana is no exception.

Aim: The aim of this study was to determine the prevalence of SCD and to assess the hemoglobin variants among patients attending Ho Teaching Hospital.

Methods: A retrospective study design was used to extract information from the Hospital Administration and Management Systems (HAMS) on the hemoglobin electrophoresis results and corresponding full blood count results of the SCD and sickle cell anemia (SCA) patients as well as patients who were asked to do Hb electrophoresis irrespective of their sickling status. Data were collected for the period January 2016 to December 2018. Sickle cell disease status was determined using the Hb genotypes from the Hb electrophoresis results. The full blood count was used to categorize the severity of anemia based on the hemoglobin concentration in the SCA and SCD patients.

Results: A total of 1,523 subjects were included in the study of which the prevalence for sickle cell disease was 16.7%. The SCD genotypes included HbS (6.2%), HbSC (7.9%), and HbSF (2.6%). Hemoglobin C disease (HbCC) constituted 0.3% out of the total prevalence of SCD. The prevalence of anemia was 99.2%, with the severest form in HbS. Also, majority of the SCD patients had severe anemia. Difference in the severity of anemia was found to be significant among both male (P=0.006) and female (P=0.004) participants with SCD.

Conclusion: Patients receiving health care at the Ho Teaching Hospital had different hemoglobin variants with HbAS recording the highest prevalence. The high incidence of hemoglobin AS implies the possibility of having an increased population of individuals with sickle cell disease in future if measures are not put in place to improve screening, counseling, and education of the public about the health threat SCD poses.

Dyslipidemia is highly prevalent in sickle cell anemia (SCA) patients and is one of the major risk factors for cardiovascular diseases induced by oxidative stress in Africa. The aim of this research was to investigate the correlation between higher atherogenic index of plasma (API) and oxidative stress in a group of patients living with SCA in Cameroon. Methods. A group of 85 homozygote SS patients (male and female) were enrolled at the Central hospital of Yaounde in Cameroon between May and October 2017. After informed consent through the signature of a consent form was obtained, the plasma was collected to determine the lipid profile while the lysate solution of RBC was used to explore some markers of oxidative stress using spectrophotometric methods. Results. Among the 85 patients included in our study, the mean age was 30 ± 5 years and the female to male ratio was 0.97. The majority of the patients (52-81%) had dyslipidaemia, and 22.4% of the patients demonstrated a higher level of atherogenic index of plasma. The patients with a higher level of total cholesterol (TC) (>240 mg/dl) and low-density lipoprotein (LDL-C) (>159 mg/dl) had at least 1,334 fold of malondialdeheyde (MDA) concentration than those with normal level. Also in the same patients, the higher atherogenic plasmatic index (API) significantly (p < 0.05) increased with the concentration of MDA. Except HDL-C, the other parameters of lipid profile had significant (p < 0.05) correlation with reduced glutathione (GsH) and total antioxidant capacity (TAC). The significant (p < 0.05) and linear regression was found between the increased MDA and higher API. Conclusion. Dyslipidemia increases oxidative stress and higher API which leads to coronary vascular disease in patients with SCA.