Book contents
- Frontmatter
- Contents
- Editor, Associate Editors, Artistic Consultant, and Contributors
- Preface
- PART I CONTEXT
- PART II ENDOTHELIAL CELL AS INPUT-OUTPUT DEVICE
- PART III VASCULAR BED/ORGAN STRUCTURE AND FUNCTION IN HEALTH AND DISEASE
- 121 Introductory Essay: The Endothelium in Health and Disease
- 122 Hereditary Hemorrhagic Telangiectasia: A Model to Probe the Biology of the Vascular Endothelium
- 123 Blood–Brain Barrier
- 124 Brain Endothelial Cells Bridge Neural and Immune Networks
- 125 The Retina and Related Hyaloid Vasculature: Developmental and Pathological Angiogenesis
- 126 Microheterogeneity of Lung Endothelium
- 127 Bronchial Endothelium
- 128 The Endothelium in Acute Respiratory Distress Syndrome
- 129 The Central Role of Endothelial Cells in Severe Angioproliferative Pulmonary Hypertension
- 130 Emphysema: An Autoimmune Vascular Disease?
- 131 Endothelial Mechanotransduction in Lung: Ischemia in the Pulmonary Vasculature
- 132 Endothelium and the Initiation of Atherosclerosis
- 133 The Hepatic Sinusoidal Endothelial Cell
- 134 Hepatic Macrocirculation: Portal Hypertension As a Disease Paradigm of Endothelial Cell Significance and Heterogeneity
- 135 Inflammatory Bowel Disease
- 136 The Vascular Bed of Spleen in Health and Disease
- 137 Adipose Tissue Endothelium
- 138 Renal Endothelium
- 139 Uremia
- 140 The Influence of Dietary Salt Intake on Endothelial Cell Function
- 141 The Role of the Endothelium in Systemic Inflammatory Response Syndrome and Sepsis
- 142 The Endothelium in Cerebral Malaria: Both a Target Cell and a Major Player
- 143 Hemorrhagic Fevers: Endothelial Cells and Ebola-Virus Hemorrhagic Fever
- 144 Effect of Smoking on Endothelial Function and Cardiovascular Disease
- 145 Disseminated Intravascular Coagulation
- 146 Thrombotic Microangiopathy
- 147 Heparin-Induced Thrombocytopenia
- 148 Sickle Cell Disease Endothelial Activation and Dysfunction
- 149 The Role of Endothelial Cells in the Antiphospholipid Syndrome
- 150 Diabetes
- 151 The Role of the Endothelium in Normal and Pathologic Thyroid Function
- 152 Endothelial Dysfunction and the Link to Age-Related Vascular Disease
- 153 Kawasaki Disease
- 154 Systemic Vasculitis Autoantibodies Targeting Endothelial Cells
- 155 High Endothelial Venule-Like Vessels in Human Chronic Inflammatory Diseases
- 156 Endothelium and Skin
- 157 Angiogenesis
- 158 Tumor Blood Vessels
- 159 Kaposi's Sarcoma
- 160 Endothelial Mimicry of Placental Trophoblast Cells
- 161 Placental Vasculature in Health and Disease
- 162 Endothelialization of Prosthetic Vascular Grafts
- 163 The Endothelium's Diverse Roles Following Acute Burn Injury
- 164 Trauma-Hemorrhage and Its Effects on the Endothelium
- 165 Coagulopathy of Trauma: Implications for Battlefield Hemostasis
- 166 The Effects of Blood Transfusion on Vascular Endothelium
- 167 The Role of Endothelium in Erectile Function and Dysfunction
- 168 Avascular Necrosis: Vascular Bed/Organ Structure and Function in Health and Disease
- 169 Molecular Control of Lymphatic System Development
- 170 High Endothelial Venules
- 171 Hierarchy of Circulating and Vessel Wall–Derived Endothelial Progenitor Cells
- PART IV DIAGNOSIS AND TREATMENT
- PART V CHALLENGES AND OPPORTUNITIES
- Index
- Plate section
146 - Thrombotic Microangiopathy
from PART III - VASCULAR BED/ORGAN STRUCTURE AND FUNCTION IN HEALTH AND DISEASE
Published online by Cambridge University Press: 04 May 2010
- Frontmatter
- Contents
- Editor, Associate Editors, Artistic Consultant, and Contributors
- Preface
- PART I CONTEXT
- PART II ENDOTHELIAL CELL AS INPUT-OUTPUT DEVICE
- PART III VASCULAR BED/ORGAN STRUCTURE AND FUNCTION IN HEALTH AND DISEASE
- 121 Introductory Essay: The Endothelium in Health and Disease
- 122 Hereditary Hemorrhagic Telangiectasia: A Model to Probe the Biology of the Vascular Endothelium
- 123 Blood–Brain Barrier
- 124 Brain Endothelial Cells Bridge Neural and Immune Networks
- 125 The Retina and Related Hyaloid Vasculature: Developmental and Pathological Angiogenesis
- 126 Microheterogeneity of Lung Endothelium
- 127 Bronchial Endothelium
- 128 The Endothelium in Acute Respiratory Distress Syndrome
- 129 The Central Role of Endothelial Cells in Severe Angioproliferative Pulmonary Hypertension
- 130 Emphysema: An Autoimmune Vascular Disease?
- 131 Endothelial Mechanotransduction in Lung: Ischemia in the Pulmonary Vasculature
- 132 Endothelium and the Initiation of Atherosclerosis
- 133 The Hepatic Sinusoidal Endothelial Cell
- 134 Hepatic Macrocirculation: Portal Hypertension As a Disease Paradigm of Endothelial Cell Significance and Heterogeneity
- 135 Inflammatory Bowel Disease
- 136 The Vascular Bed of Spleen in Health and Disease
- 137 Adipose Tissue Endothelium
- 138 Renal Endothelium
- 139 Uremia
- 140 The Influence of Dietary Salt Intake on Endothelial Cell Function
- 141 The Role of the Endothelium in Systemic Inflammatory Response Syndrome and Sepsis
- 142 The Endothelium in Cerebral Malaria: Both a Target Cell and a Major Player
- 143 Hemorrhagic Fevers: Endothelial Cells and Ebola-Virus Hemorrhagic Fever
- 144 Effect of Smoking on Endothelial Function and Cardiovascular Disease
- 145 Disseminated Intravascular Coagulation
- 146 Thrombotic Microangiopathy
- 147 Heparin-Induced Thrombocytopenia
- 148 Sickle Cell Disease Endothelial Activation and Dysfunction
- 149 The Role of Endothelial Cells in the Antiphospholipid Syndrome
- 150 Diabetes
- 151 The Role of the Endothelium in Normal and Pathologic Thyroid Function
- 152 Endothelial Dysfunction and the Link to Age-Related Vascular Disease
- 153 Kawasaki Disease
- 154 Systemic Vasculitis Autoantibodies Targeting Endothelial Cells
- 155 High Endothelial Venule-Like Vessels in Human Chronic Inflammatory Diseases
- 156 Endothelium and Skin
- 157 Angiogenesis
- 158 Tumor Blood Vessels
- 159 Kaposi's Sarcoma
- 160 Endothelial Mimicry of Placental Trophoblast Cells
- 161 Placental Vasculature in Health and Disease
- 162 Endothelialization of Prosthetic Vascular Grafts
- 163 The Endothelium's Diverse Roles Following Acute Burn Injury
- 164 Trauma-Hemorrhage and Its Effects on the Endothelium
- 165 Coagulopathy of Trauma: Implications for Battlefield Hemostasis
- 166 The Effects of Blood Transfusion on Vascular Endothelium
- 167 The Role of Endothelium in Erectile Function and Dysfunction
- 168 Avascular Necrosis: Vascular Bed/Organ Structure and Function in Health and Disease
- 169 Molecular Control of Lymphatic System Development
- 170 High Endothelial Venules
- 171 Hierarchy of Circulating and Vessel Wall–Derived Endothelial Progenitor Cells
- PART IV DIAGNOSIS AND TREATMENT
- PART V CHALLENGES AND OPPORTUNITIES
- Index
- Plate section
Summary
The fundamental lesion in idiopathic thrombotic thrombocytopenic purpura (TTP), sporadic hemolytic-uremic syndrome (HUS), and some forms of familial HUS/TTP is a thrombotic microangiopathy (1–3).Hyaline thrombi, composed predominantly of platelets and accompanied by localized endothelial cell (EC) activation, proliferation, and detachment, are found exclusively in the microvasculature of involved tissues (1). These thrombi subserve the clinical manifestations of both disorders. Initial treatment consists of plasmapheresis and plasma infusions, often requiring over 20 liters of plasma. Failure to institute prompt plasma exchange invariably results in death from renal failure, stroke, myocardial infarction, or other thrombotic events.
Although TTP is recognized classically by the pentad of thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurological signs, renal abnormalities, and fever, and HUS by a triad of signs – the hemolytic anemia and fever seen in TTP plus prominent renal disease (4) – pathological changes overlap significantly. Sporadic HUS patients often have multiple organ involvement, including dermal, ocular, cardiac, gastrointestinal, and neurological changes, similar to that seen in TTP (1–3). In one study, cerebral microthrombi were found in 13 of 26 HUS patients at autopsy; reciprocally, renal dysfunction occurs in 40% to 80% of TTP cases (3). Only two organs are absolutely or relatively spared in classic TTP/sporadic HUS: The lung is virtually never involved (5), and the liver is an infrequent site of pathology (2).
It is critical – in terms of pathophysiology and selection of appropriate therapeutic interventions – to distinguish between two clinical syndromes commonly labeled HUS. The epidemic type, occurring primarily in children younger than 5 years of age and typically preceded by bloody diarrhea, is linked to the shiga-like verocytotoxins (4).
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- Endothelial Biomedicine , pp. 1337 - 1343Publisher: Cambridge University PressPrint publication year: 2007