Effectiveness of Nurse Led Intervention on Health Related Quality of Life among Children with Sickle Cell Disease in Oman: A Pilot StudyRead the full article
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Prognostic Impact of Lymphoid Enhancer Factor 1 Expression and Serum Galectin.3 in Egyptian AML Patients
Background. Deregulation of the Wnt signaling pathway had a role in haematological malignancies. Previous studies reported that lymphoid enhancer factor 1 (LEF1) expression and serum Galectin-3 level could affect clinical parameters and outcome in acute myeloid leukemia patients, but as far as we know, no study has addressed their combined effect on AML patients. Aim. We studied the expression of LEF1 by real-time qPCR and measured serum level of Gal.3 by ELISA technique in peripheral blood of 69 AML patients and correlated it with different clinicopathological criteria of patients, response, PFS and OS. Results. We found high expression (LEF1high) was associated with better OS () and EFS () compared to LEF1low, low serum Gal.3 level had better OS () and EFS () compared to high serum Gal.3 level. LEF1high less likely to carry a FLT3-ITD () compared to LEF1low patient, also LEF1high characterized by favorable risk () than LEF1low patients. While patients with higher Gal-3 levels characterized by poor risk () than lower Gal.3 lels, also more likely to carry a FLT3-ITD with borderline significance (). Combined LEF1high/Gal.3 low patients had lower baseline blast percentages (), favorable risk (), less likely to carry FLT3-ITD (), higher CR rate (), shorter time to CR (0.001) than other groups. Among high Gal.3 level group, LEF1high expression improved OS and EFS (20 and 15 months respectively) vs LEF1low expression (13 and 8 months respectively). Conclusion. We conclude that high LEF1 expression was a favorable prognostic marker which can define AML patient risk and outcome independent from assessing the serum galectin.3 level.
Study of Frequency and Characteristics of Red Blood Cell Alloimmunization in Thalassemic Patients: Multicenter Study from Palestine
Background. β-Thalassemia is a common inherited hemolytic disorder in Palestine. Red blood cell (RBC) transfusion is the principal treatment but it may cause RBC alloimmunization. This study was conducted to determine the prevalence and characteristics of RBC alloimmunization among thalassemic patients in northern governorates of Palestine. Methods. A prospective multicenter observational study was conducted in the thalassemia transfusion centers in the northern governorates of Palestine. The study included 215 thalassemia patients who received regular blood transfusions. Clinical and transfusion records of patients were examined. Antibody screening and identification was conducted using the microcolum gel technique. Results. Two hundred fifteen patients were included in the study. More than half (52.1%) of the patients were males. The median age of patients was 18 years (range: 12–24 years). The most frequent blood group was A (40.5%). Alloantibodies were detected in 12.6% of patients. Anti-D (33.3%), anti-K (25.9%) and anti-E (14.8%) were the most commonly isolated antibodies. There was no association between age, sex, starting age of transfusion, number of transfused units, history of splenectomy and alloimmunization. Conclusions. Anti-Rh and anti-K antibodies were common among this cohort of patients. Age, sex, starting age of transfusion, number of transfused units, and history of splenectomy could not predict the occurrence of alloimmunization.
Coexistence of Myeloid and Lymphoid Neoplasms: A Single-Center Experience
The coexistence of a myeloid and a lymphoid neoplasm in the same patient is a rare finding. We retrospectively searched the records of the Hematology Division of the Second Department of Internal Medicine and Research Institute at Attikon University General Hospital of Athens from 2003 to 2018. Nine cases have been identified in a total of 244 BCR-/ABL1- negative MPN and 25 MDS/MPN patients and 1062 LPD patients referred to our institution between 2003 and 2018. Each case is distinct in the diversity of myeloid and lymphoid entities, the chronological occurrence of the two neoplasms, and the patient clinical course. All of them exhibit myeloproliferative (6 JAK2 V617F-positive cases) and lymphoproliferative features, with 1 monoclonal B-cell lymphocytosis (MBL), 3 B-chronic lymphocytic leukemias (B-CLL), 3 B-non-Hodgkin lymphomas (B-NHL), 1 multiple myeloma (MM), and 1 light and heavy deposition disease (LHCDD), while in three cases myelodysplasia is also present. The challenges in identifying and dealing with these rare situations in everyday clinical practice are depicted in this article.
Prevalence of Anemia and Associated Factors among Newly Diagnosed Patients with Solid Malignancy at Tikur Anbessa Specialized Hospital, Radiotherapy Center, Addis Ababa, Ethiopia
Background. Anemia is a common finding in cancer, which is caused by many factors. It is a major cause of morbidity in cancer patients, worsens disease status and impairs treatment outcome; however, little is known about the prevalence of anemia and associated factors among cancer patients during diagnosis in developing countries like Ethiopia. In response to this, we have conducted research with the aim of assessing the prevalence of anemia and associated factors among newly diagnosed patients with solid malignancy at Tikur Anbessa Specialized Hospital (TASH), Radiotherapy center, Addis Ababa, Ethiopia. Methods. Descriptive cross-sectional study was conducted from April to May 2014. A total of 422 newly diagnosed patients with solid malignancy attending Radiotherapy center, TASH were enrolled to assess anemia prevalence and associated factors. Data were coded, entered and analyzed using SPSS version16. Using logistic regression, chi squares, Odds ratio and 95% confidence intervals were computed to measure strength of association between variables. -value < 0.05 was taken as statistically significant. Result. Out of 422 respondents, 285 (68%) were females and 153 (36%) of respondents fell into 35–49 age group with age range between 18 and 80 years and the median age of 45. Magnitude of solid cancers was gynecologic (28.9%), breast (22.7%), nasopharyngeal carcinoma (NPC) (7.6%), colorectal (7.1%), sarcoma (6.9%), head and neck (4.5%), thyroid (3.3%), hepatoma (1.9%), and others (17.1%). The overall prevalence of anemia across different tumor was 23% and higher anemia prevalence was noted in gynecologic (37.7%) and colorectal carcinomas (26.7%). The majority of the anemic patients (68%) remained untreated for anemia. The mean trigger hemoglobin for transfusion was 7.7 g/dl. About 83.5% of anemia was mild to moderate type. Performance status (AOR = 3.344; 95% CI 1.410–7.927) and bleeding history (AOR = 3.628; 95% CI 1.800–7.314) showed statistically significant association with occurrence of anemia with -value < 0.05. Conclusion. Among solid cancers, gynecologic cancer remained the dominant one. Anemia prevalence was 23% in general, in which gynecologic and colorectal cancers were more prevalent. ECOG performance status and bleeding history showed a statistically significant association with the occurrence of anemia.
Management of Adult Patients with Primary Immune Thrombocytopenia (ITP) in Clinical Practice: A Consensus Approach of the Spanish ITP Expert Group
Background and Objective. Diagnosis and management of primary immune thrombocytopenia (ITP) have changed dramatically in the last decade. The aim of the study was to obtain information about the opinion of the Spanish ITP Group (GEPTI) members regarding the best clinical practices for diagnosis and management of adult patients with ITP. Materials and Methods. A two-round Delphi method was carried out by sending to 129 experts a 90-item questionnaire developed by 11 specialists, with a 4-point Likert scale (“never,” “sometimes,” “frequently,” and “always”) for the assessment of responses. Results. Forty out of the 129 experts participated in the survey (participation rate 30.2%) and 39 completed the questionnaire (response rate 97.5%). Salient consensus points included the following: the need to indicate workup studies from a sustained platelet count < 100 x 109/L in the absence of a clear etiology; bone marrow aspiration in elderly patients with suspected ITP; beginning treatment in asymptomatic patients with a platelet count < 20 x 109/L; not exceeding 6-7 weeks of corticosteroid therapy; switching from corticosteroids to one thrombopoietin receptor agonist (TRA); switching to other TRA or other options as combinations of them with immunosuppressive drugs in case of failure; how to reduce tapering TRA; treating patients with symptomatic persistent ITP and platelet count > 20 x 109/L; and considering mucosal or severe bleeding as a basic criterion for hospital admission. Conclusions. The present consensus document provides a reference framework for the management of patients with ITP in clinical practice.
Quantifying the Levels of Knowledge, Attitude, and Practice Associated with Sickle Cell Disease and Premarital Genetic Counseling in 350 Saudi Adults
Our study aims to observe the levels of knowledge, attitude, and practice (KAP) associated with sickle cell disease (SCD) and premarital genetic counseling (PMGC) in 351 Saudi adults. The relationships between KAP levels and sociodemographic characteristics (age, gender, marital status, and educational level) were observed. The study was conducted in King Khalid University Hospital between February 21, 2017, and March 7, 2018. A total of 351 Saudi participants attending the primary care clinic were selected using convenience sampling and were given a self-administered questionnaire. Overall, the 351 participants had the best attitude (41% scoring “good”), followed by knowledge (28.8%), and, lastly, practice (19.1%). Out of the sociodemographic characteristics, age group was the most statistically significant in all the three categories (knowledge, attitude, and practice). The > 50-year age group performed the worst in all the three categories. Despite the advancements in public healthcare measures in Saudi Arabia, our study revealed that there are still many gaps to be filled regarding the knowledge, attitude, and practice associated with SCD and PMGC.