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Oxford handbook of clinical haematology
Providing essential information needed in clinical practice for the diagnosis and management of patients with blood disorders, this handbook covers haematological investigations and their interpretation, and commonly used protocols.
Book
Philadelphia Chromosome as a Clinically Favorable Prognostic Factor of B-cell Acute Lymphoblastic Leukemia/Lymphoblastic Lymphoma in Transplant-Ineligible Elderly Patients in the Era of Molecular-Targeted Therapy
Background and objective There is scarce data on the treatment outcomes of B-cell acute lymphoblastic leukemia/lymphoblastic lymphoma (B-ALL/LBL) in elderly patients in the era of tyrosine kinase inhibitors (TKIs), blinatumomab, and inotuzumab ozogamicin. In light of this, we aimed to address this gap in data by conducting this retrospective study. Methods Treatment outcomes were retrospectively evaluated by using data from transplant-ineligible patients aged 65 years or older with newly diagnosed B-ALL/LBL (n=29) at two hospitals in Oita, Japan between 2013 and 2023. Results The median age of the cohort was 72 (65-88) years, and 10 patients were male; 17 patients had Philadelphia chromosome (Ph)-positive ALL, and the others had Ph-negative ALL. Dasatinib combined with prednisolone was the most common induction therapy for Ph-positive ALL (88.2%). Complete response (CR) was achieved in 93.1%, and the CR rate did not differ significantly between Ph-positive ALL (100%) and Ph-negative ALL (83.3%) (p=0.16). The median observation period was 1.52 (range: 0.03-8.98) years. Overall survival (OS) and event-free survival (EFS) were significantly longer in Ph-positive ALL patients than in Ph-negative ALL patients on univariate analysis (OS: p=0.011, EFS: p=0.041). Multivariate analyses showed that the presence of Ph was significantly and independently associated with longer OS [hazard ratio (HR): 0.29, 95% confidence interval (CI): 0.10-0.87, p=0.027] and EFS (HR: 0.34, 95% CI: 0.12-0.91, p=0.03). There was no difference in relapse-free survival (RFS); 13 patients (76.5%) with Ph-positive ALL were treated with ponatinib (salvage therapy, n=7; consolidation or maintenance therapy in CR, n=6). Six of seven patients (85.7%) with ponatinib salvage therapy achieved CR, and all six patients treated with ponatinib consolidation or maintenance therapy retained CR at the last follow-up. Six patients (Ph-positive ALL: n=4; Ph-negative ALL: n=2) were treated with blinatumomab, including salvage therapy for primary refractory or relapse (n=3), and consolidation therapy due to intolerance to conventional chemotherapies (n=3). Two of three patients with blinatumomab salvage therapy achieved CR, and all three patients with blinatumomab consolidation therapy maintained CR in follow-up. Two patients (Ph-positive ALL: n=1; Ph-negative ALL: n=1) were treated with inotuzumab ozogamicin for relapsed or refractory ALL. A patient with Ph-positive ALL for the third relapse achieved CR, which was sustained for three years. The other patient with Ph-negative ALL for primary refractory achieved CR but relapsed after the fourth course of inotuzumab ozogamicin. Conclusions Elderly patients with Ph-positive ALL showed significantly longer OS and EFS than those with Ph-negative ALL in the era of molecular-targeted therapy.
Journal Article
A beginner's guide to blood cells
A Beginner's Guide to Blood Cells is a straightforward text for those starting to examine blood films for the first time. No prior knowledge is assumed as the descriptions of haematological conditions start with normal cells and explanations of the terms commonly used to describe them. This leads on to abnormal findings. A Beginner's Guide to Blood Cells: outlines the basics of diagnostic haematology; stresses the skills required to diagnose diseases of the blood by morphological analysis; describes how to analyse a blood film, how to recognize any abnormality present and how to interpret abnormalities; explains the clinical relevance of haematology tests in patient care; gives a simple explanation of the full blood count and a table of normal ranges; and tests understanding with a self-assessment section. A Beginner's Guide to Blood Cells is ideal for a diverse group of practitioners who need a concise overview of diagnostic haematology.
Aplastic Anemia: Demographic and Clinical Characteristics in Costa Rica
Background Aplastic anemia (AA) is a rare and heterogeneous hematological disorder defined as pancytopenia with hypocellular bone marrow in the absence of abnormal infiltration or medullary fibrosis. Various causes of AA have been identified, such as autoimmune factors, bone marrow injuries, viral infections, and genetic disorders. The symptoms of AA are directly linked to pancytopenia and the most common are fatigue, recurrent infections, and bleeding problems. The treatment of AA varies according to the severity of the disease and includes immunosuppressive therapies and bone marrow transplantation. This study aims to identify the most relevant social, clinical, and demographic characteristics of patients with AA in Costa Rica. Methodology A retrospective, observational study was conducted in Costa Rica by reviewing the medical records of patients diagnosed with AA in the main hospitals of the Costa Rican Social Security Health Fund (CCSS, by its acronym in Spanish). A total of 109 patients who were evaluated between 2016 and 2018 were identified. Sociodemographic, clinical, and treatment information was collected for these patients in a database that was analyzed using statistical programs such as SPSS Statistics (version 24) and GraphPad Prism (version 8). Results Most patients were male (56%) with an average age of 32 years. Patients were classified according to the severity of the disease, and a higher mortality at 60 months was observed in those with very severe AA and in patients over 65 years old. The most commonly used first-line treatment was the combination of rabbit antithymocyte globulin (ATG) and cyclosporine (42.9%). Patients who required a greater number of blood transfusions had a more severe disease. Further, 46 patients requiring a second line of treatment were identified, and the most common treatment in this group was the combination of ATG with eltrombopag in 19.6% of the patients. The study results present the sociodemographic and clinical characteristics of patients with AA in Costa Rica. The lack of identification of a common external factor that may influence the development of the disease is highlighted. Treatment with rabbit ATG and cyclosporine demonstrated a good response in patients. The availability and cost of treatments are important considerations, especially in developing countries. Conclusions The study highlights significant progress in the understanding and treatment of AA in the Costa Rican context. The results support the efficacy of the combination of antibodies and cyclosporine as a therapeutic option. The importance of adapting treatments to the characteristics of the local population is emphasized, along with the need for further research to improve long-term outcomes.
Journal Article
Treatment of Secondary Hemophagocytic Lymphohistiocytosis Associated With Diffuse Large B-cell Lymphoma Using Loncastuximab Tesirine As Lymphoma-Directed Therapy
Three critically ill, chemotherapy-refractory patients with diffuse large B-cell lymphoma (DLBCL) received loncastuximab tesirine in conjunction with standard therapies for secondary malignancy-associated hemophagocytic lymphohistiocytosis (Mal-HLH). All patients were treated inpatient, with one requiring intubation on the day of administration. Each patient had an H-score >238, indicating a >98% probability of HLH. A significant reduction in ferritin levels was observed in two patients, and one patient achieved a complete response (CR). Loncastuximab tesirine demonstrated promise in managing Mal-HLH where previous treatments had failed. This study suggests that loncastuximab tesirine exhibits favorable activity and should be considered a valuable addition to the treatment options for Mal-HLH driven by DLBCL.
Journal Article