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A review on disease modifying pharmacologic therapies for sickle cell disease.
Ann Hematol
January 2025
Department of Internal Medicine, Section of Hematology/Oncology, University of Missouri-Kansas City, Kansas City, MO, 64108, USA.
Sickle cell disease (SCD) is an inherited hematologic disease caused by sickle hemoglobin as the predominant RBC hemoglobin or by sickle hemoglobin in combination with other abnormal β-hemoglobin variants like HbC, HbD and others. Sickling of erythrocytes under deoxygenated conditions is the basis of inflammatory and thrombotic cascades which result in multiple serious complications, leading to early morbidity and mortality. While HLA-matched allogeneic bone marrow transplantation is potentially curative, it has considerable limitations due to potential severe toxicities.
View Article and Find Full Text PDFMusculoskeletal complications in sickle cell disease: Pathophysiology, diagnosis and management.
Best Pract Res Clin Rheumatol
January 2025
ICMR-National Institute of Research in Tribal Health, Jabalpur, India; Academy of Scientific and Innovative Research (AcSIR), Ghaziabad, India; Model Rural Health Research Unit, Jheet, Durg, India. Electronic address:
Sickle cell disease (SCD) is a mono-genic disorder causing chronic hemolysis, anemia, and vaso-occlusion, leading to musculoskeletal complications such as osteonecrosis, osteoporosis, and bone fractures affecting 50-70% SCD patients. These complications result from a complex interplay of genetic and physiological factors, including abnormal hemoglobin production, chronic inflammation, and oxidative stress. This review discusses the pathophysiology, pre-clinical symptoms, and clinical manifestations of musculoskeletal complications in SCD, as well as current treatment options, including pharmacological interventions, surgical procedures, and bone marrow transplantation.
View Article and Find Full Text PDFRelationship between additional mutations at diagnosis and treatment response in patients with essential thrombocythemia.
Blood Adv
January 2025
Univ Angers, Nantes Université, CHU Angers, Inserm, CNRS, CRCI2NA, F-49000, Angers, France, ANGERS, France.
Patients with essential thrombocythemia (ET) have a chronic evolution with a risk of hematological transformation associated with a dismal outcome. Since patients with resistance or intolerance have an adverse prognosis, it is important to identify which patient will respond to first-line treatment. We therefore aim to describe the association between additional mutations and response to first-line treatment in patients with ET.
View Article and Find Full Text PDFPARADIGM-PV: a randomized, multicenter phase 4 study to assess the efficacy and safety of ropeginterferon alfa-2b in patients with low- or high-risk polycythemia vera.
Ann Hematol
January 2025
Department of Medicine, School of Clinical Medicine, LKS Faculty of Medicine, the University of Hong Kong, Hong Kong, China.
Polycythemia vera (PV) is characterized by clonal hematopoietic stem or progenitor cells with constitutively active somatic mutation(s) in the Janus kinase 2 gene. Phlebotomy (Phl) and aspirin are often used alone for low-risk PV patients. However, data from the Low-PV study demonstrated that Phl and aspirin may not be adequate for patients.
View Article and Find Full Text PDFKnowledge, Attitude, and Practice of Patients Suffering From Sickle Cell Disease in an Endemic Zone.
J Pediatr Hematol Oncol
January 2025
Medical Oncology, Haemato-Oncology, BMT, Sarvodaya Hospital, Faridabad, Haryana, India.
Background And Objective: Sickle cell disease (SCD) is a genetically inherited disorder that is associated with morbidity and mortality.
Methods: This cross-sectional study was conducted on patients diagnosed with SCD to evaluate the knowledge, attitude, and practice of patients/guardians using a pretested questionnaire.
Results And Discussion: Of the 111 participants, 56 (50.
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