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50 Results Found for “von Willebrand Factor”
The first primary microangiopathic haemolytic anaemia was identified as thrombotic thrombocytopenic purpura (TTP) by Dr Moschowitz in 1924. At that time, the classic pentad of clinical features, still used today, included fever, erythrocyte fragmentation, thrombocytopenia, kidney injury and neurologic injury.1 It ...
In the latest edition of touchREVIEWS in Oncology & Haematology, we are pleased to present a collection of articles that delve into the latest research and advancements in the field. From innovative therapies and genetic treatments to analyses of digital ...
Severe haemophilia A (HA), defined as baseline factor VIII (FVIII) levels of <1%, results in traumatic and spontaneous bleeding episodes, which occur primarily in the joints, in addition to the muscles, soft tissue and the central nervous system. Prophylactic treatment ...
In December 2019, an outbreak of pneumonia of unknown aetiology was observed in the Chinese city of Wuhan, capital of Hubei province. By January 2020, this outbreak was attributed to a novel virus classified as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the ...
Amyloidosis is a group of diseases resulting from intracellular and extracellular deposition of insoluble abnormal amyloid fibrils, which alters the normal function of tissues. Glycosaminoglycans, apolipoprotein-E and serum amyloid P component comprise 10% of deposits, while amyloid fibrils formed by misfolded ...
At the 61st ASH Annual Meeting & Exposition, editorial board member Pier Mannuccio Mannucci discusses new therapies for von Willebrand disease. To view Part 1, click here Questions 1. What are the advantages of von Willebrand factor (VWF)-only products? (0:05) 2. What is ...
Therapeutic plasma exchange (TPE) is an extracorporeal technique that involves separating a large volume of a patient’s plasma from the cellular components of the blood and replacing it with appropriate fluids.1 In patients with conditions that are induced and/...
In haematologic malignancies with an unfavourable prognosis, high-dose chemotherapy followed by haematopoietic stem cell transplantation (HSCT) is the only realistic curative therapeutic option.1 Allogeneic and autologous haematopoietic stem cells are routinely used for transplantation. Allogeneic HSCT is associated with a ...
Early diagnosis of rare haematological diseases Rare haematological diseases often present ‘in disguise’, with nonspecific symptoms such as pancytopaenia, decreased circulating levels of one or two cell subsets and splenomegaly. Gaucher disease (GD) provides an excellent paradigm when considering challenges ...
Haematopoietic stem cell transplantation (HSCT) has become the standard of care for many haematological malignancies, selected solid tumours and some non-malignant disorders.1 However, it can be associated with serious complications, in particular, veno-occlusive disease (VOD). Approximately 14 % of HSCT patients develop ...
Classification The two basic forms of thrombotic microangiopathies, excluding disseminated intravascular coagulation (DIC), include thrombotic thrombocytopenic purpura (TTP), and hemolytic uremic syndrome (HUS). Early historic reports noted the presence of hemolytic anemia and thrombocytopenia in both disorders and suggested differentiation ...
Thrombotic thrombocytopenic purpura (TTP) is an acute prothrombotic disorder resulting from a deficiency of the von Willebrand factor cleavage protease ADAMTS13.1 The enzyme ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats) cleaves the peptide bond between Tyr-842 and Met-843 ...
Phenotypic diversity within the β-thalassaemia syndromes has traditionally received considerable interest, with several molecular and environmental modifiers of disease severity so far described.1 Patients with transfusion-dependent β-thalassaemia major (TM) suffer the most severe form and show the highest mortality rates.1 ...
Phenotypic diversity within the β-thalassemia syndromes has traditionally received considerable interest, with several molecular and environmental modifiers of disease severity so far described.1 Patients with transfusion-dependent β-thalassemia major (TM) suffer the most severe form and show the highest mortality rates.1 ...
Sickle cell disease (SCD) is a common hemolytic anemia caused by a single gene mutation in the β-subunit of hemoglobin (Hb),1 affects millions of people worldwide, and is associated with significant morbidity and mortality. An estimated 83,000 US citizens have been ...
Introduction to Decision Analysis Some statisticians might question whether Hamlet could have been saved by clinical decision analysis. In his famous soliloquy, Hamlet pondered: “To be or not to be—that is the question.” With some refinement, this existential questioning ...
Human cytomegalovirus (CMV) is a common human pathogen that infects the majority of the world’s population. CMV is a large species-specific virus that co-evolved with its host for many thousands of years. Since CMV adapted to persist in immunocompetent ...
When a blood vessel is injured, platelets adhere to the exposed subendothelium (platelet adhesion). The platelets are activated (platelet activation) and secrete their granule contents (platelet secretion). The granule contents include platelet agonists (adenosine diphosphate [ADP] and serotonin) that, by ...
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