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Fast Facts: Thrombotic Thrombocytopenic Purpura
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 their 40s. It is also clear that the prompt delivery of plasma exchange saves lives. 'Fast Facts: Thrombotic Thrombocytopenic Purpura' sets out, in a clear and accessible format, the steps to suspecting, diagnosing and treating this potentially devastating disease. These steps are complemented by clear descriptions of the disease mechanism and epidemiology. Differential diagnosis, which is of the utmost importance for this disease, is explored in detail. Contents: ‧ Disease overview ‧ Clinical presentation ‧ Differential diagnosis ‧ Laboratory findings and diagnosis ‧ Management
- Cover
- Title Page
- Copyright
- Contents
- List of abbreviations
- Introduction
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Disease overview
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Epidemiology
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Etiology
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Clinical presentation
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Classic presentation of acute TTP
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Atypical presentations in adulthood
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Childhood and adolescent onset
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Association with other autoimmune diseases
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Congenital (hereditary) TTP
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End-organ damage in cTTP
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Subacute features of cTTP
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Presentation in pregnancy
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Differential diagnosis
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Immune-mediated TTP
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STEC-HUS (typical or diarrhea-associated HUS)
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Atypical HUS
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Drug-induced TMA
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Secondary TMA
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Systemic causes of anemia and/or thrombocytopenia that mimic TMA
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Differential diagnostic approach
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Laboratory findings and diagnosis
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Diagnosing TTP
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Confirming the diagnosis
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Screening for severe ADAMTS13 deficiency
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Management
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Immune-mediated TTP
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Congenital TTP
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- Useful resources
- Index
- 出版地 : 德國
- 語言 : 德文
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