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Fast Facts for Patients: 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. Table of Contents: ‧ Disease overview ‧ Clinical presentation ‧ Differential diagnosis ‧ Laboratory findings and diagnosis ‧ Management
- Cover
- Contents
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1. What is thrombotic thrombocytopenic purpura (TTP)?
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Blood cells
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Blood clotting
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What happens in TTP?
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Types of TTP
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Immune TTP (iTTP)
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Congenital TTP (cTTP)
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How is cTTP inherited?
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2. Symptoms
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Common symptoms
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Long-term symptoms
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3. How is TTP treated?
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Monitoring your treatment
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Platelet counts
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ADAMTS13 levels
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Immune TTP (iTTP) treatment: plasma exchange
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Possible side effects
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Practical tips
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Immune TTP (iTTP) treatment: medications that suppress the immune system
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Steroids
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Possible side effects
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Practical tips
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Rituximab/anti-CD20 therapy
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Possible side effects
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Practical tips
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Mycophenolate mofetil (MMF)
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Possible side effects
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Practical tip
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Velcade®
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Possible side effects
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Other treatments
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Immune TTP (iTTP) treatment: medications that prevent blood clots (thrombosis)
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Caplacizumab
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Possible side effects
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Aspirin and low-molecular-weight heparin
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Immune TTP (iTTP) treatment: other supporting medications/treatments
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Folic acid
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Omeprazole, lansoprazole and ranitidine
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Blood transfusion
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Treatment for congenital TTP (cTTP)
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Plasma infusion
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4. Clinical trials
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Recombinant ADAMTS13
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UK TTP registry
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- 5. Psychological support and neuropsychological testing
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6. Going home after TTP treatment
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Practical tips
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Medications after discharge
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What to do if you feel unwell
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Follow-up appointments
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TTP in the long term
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Frequently asked questions about TTP
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- Useful resources
- Glossary
- Copyright
- 出版地 : 德國
- 語言 : 德文
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