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Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of the blood coagulation system. In most cases, a lack of the ADAMTS13 enzyme leads to an accumulation of ultra-large von Willebrand factor molecules in the plasma which, in turn, initiate the formation of microscopic thromboses in small blood vessels. TTP is a medical emergency. Timely diagnosis and urgent and effective management are vital – mortality in those untreated is in the region of 90%. The understanding of TTP pathogenesis has increased markedly in recent decades. It is now known that TTP is acquired (immunemediated) or congenital, and that the most common type – the acquired form – predominantly affects women in t...
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of the blood coagulation system. In most cases, a lack of the ADAMTS13 enzyme leads to an accumulation of ultra-large von Willebrand factor molecules in the plasma which, in turn, initiate the formation of microscopic thromboses in small blood vessels. TTP is a medical emergency. Timely diagnosis and urgent and effective management are vital – mortality in those untreated is in the region of 90%. The understanding of TTP pathogenesis has increased markedly in recent decades. It is now known that TTP is acquired (immunemediated) or congenital, and that the most common type – the acquired form – predominantly affects women in t...
Thrombotic thrombocytopenic purpura is a life-threatening occlusive disorder of the microcirculation that is characterized by systemic platelet agglutination, organ ischaemia, severe thrombocytopenia and fragmentation of red blood cells. In the opening study included in Thrombotic Thrombocytopenic Purpura: Causes, Diagnosis and Treatment, the authors analyze the principal risk factors and causes of this disorder.Thrombotic thrombocytopenic purpura is diagnosed using standard laboratory tests: in addition to microangi-opathic hemolytic anemia and consumption thrombocytopenia, classical parameters for hemolysis show an elevated reticulocyte count, an undetectable serum haptoglobin concentratio...
According to the Autoimmune Diseases Coordinating Committee (ADCC), between 14.7 and 23.5 million people in the USA – up to eight percent of the population are affected by autoimmune disease. Autoimmune diseases are a family of more than 100 chronic, and often disabling, illnesses that develop when underlying defects in the immune system lead the body to attack its own organs, tissues, and cells. In Handbook of Autoimmune Disease, the editors have gathered in a comprehensive handbook a critical review, by renowned experts, of more than 100 autoimmune diseases, divided into two main groups, namely systemic and organ-specific autoimmune diseases. A contemporary overview of these conditions with special emphasis on diagnosis is presented. Each chapter contains the essential information required by attending physicians as well as bench scientists to understand the definition of a specific autoimmune disease, the diagnostic criteria, and the treatment.
This reference presents detailed discussions of the history, pathology, pathophysiology, and approaches to treatment of the complicated, constantly evolving syndromes known as thrombotic thromocytopenic purpura (TTP), from many different points of view. Hemolytic Uremic syndrome and Thrombotic Thrombocytopenic Purpura offers: extensive analyses of the relationship between HUS and TTP; epidemiological studies of HUS from the UK, Canada, Asia, South Africa and Argentina; investigations of non-renal complications of HUS; perspectives on atypical HUS and post-transplantation HUS; delineations of the association between verotoxin and HUS, HUS and pregnancy, and HUS and cancer and cancer tharapy; ...
This text provides a concise yet comprehensive overview of the most common autoimmune cytopenias affecting adults and children. The book is divided into four sections, each of which focuses on a major autoimmune cytopenia. The first section features background, pathophysiology, presentation, evaluation, and treatment strategies for immune thrombocytopenia (ITP), the most common cause of antibody-mediated platelet destruction. The second section reviews common forms and treatment strategies for autoimmune hemolytic anemia (AIHA), including a chapter dedicated specifically to Evans Syndrome. The third section comprehensively reviews the pathophysiology, diagnosis and current management approac...
This book contains a variety of medical case studies from actual patients presenting to the emergency department. It includes not only typical cases that present to the ED but also less common, yet very important cases that one can't afford to miss. Each chapter begins with a case – or set of cases with typical and atypical aspects – of the disease in question. This is followed by high-value learning points on the condition with introductory/background points, physiology and pathophysiology of the disease, how to make the diagnosis, and finally how to initiate treatment. The cases provide expert discussion with tips and tricks, personal experience with management of each of the cases, an...
The second edition of a succinct and portable text reviewing the clinical approach to emergency medicine and critical care.