Biomaterial Database

Thrombotic thrombocytopenic purpura. I. Pathophysiology and clinical manifestations. 1984

R H Sills

Thrombotic thrombocytopenic purpura is an uncommon, life-threatening disorder that affects older children and adolescents as well as adults. A variety of theories have been proposed to explain its clinical and pathologic manifestations, but the pathophysiology remains poorly understood. It is not even clear whether this disease primarily affects the endothelial cell, the platelet, or both. Most patients have no discernable predisposition to this disease. Our failure to define the pathophysiology of thrombotic thrombocytopenic purpura adequately has hampered our ability to design rational and consistently successful therapy. The present knowledge of this pathophysiology is discussed in detail. The high mortality of this disease necessitates rapid diagnosis so that therapy can be instituted as quickly as possible. The clinical manifestations and diagnostic criteria of thrombotic thrombocytopenic purpura are therefore reviewed.

UI	MeSH Term	Description	Entries
D007674	Kidney Diseases	Pathological processes of the KIDNEY or its component tissues.	Disease, Kidney,Diseases, Kidney,Kidney Disease
D008297	Male		Males
D009461	Neurologic Manifestations	Clinical signs and symptoms caused by nervous system injury or dysfunction.	Neurologic Deficits,Neurologic Signs and Symptoms,Focal Neurologic Deficits,Manifestations, Neurologic,Manifestations, Neurological,Neurologic Dysfunction,Neurologic Findings,Neurologic Manifestation,Neurologic Signs,Neurologic Symptoms,Neurological Manifestations,Deficit, Focal Neurologic,Deficit, Neurologic,Deficits, Focal Neurologic,Deficits, Neurologic,Dysfunction, Neurologic,Dysfunctions, Neurologic,Finding, Neurologic,Findings, Neurologic,Focal Neurologic Deficit,Manifestation, Neurologic,Manifestation, Neurological,Neurologic Deficit,Neurologic Deficit, Focal,Neurologic Deficits, Focal,Neurologic Dysfunctions,Neurologic Finding,Neurologic Sign,Neurologic Symptom,Neurological Manifestation,Sign, Neurologic,Signs, Neurologic,Symptom, Neurologic,Symptoms, Neurologic
D010972	Platelet Activating Factor	A phospholipid derivative formed by PLATELETS; BASOPHILS; NEUTROPHILS; MONOCYTES; and MACROPHAGES. It is a potent platelet aggregating agent and inducer of systemic anaphylactic symptoms, including HYPOTENSION; THROMBOCYTOPENIA; NEUTROPENIA; and BRONCHOCONSTRICTION.	AGEPC,Acetyl Glyceryl Ether Phosphorylcholine,PAF-Acether,Phosphorylcholine, Acetyl Glyceryl Ether,1-Alkyl-2-acetyl-sn-glycerophosphocholine,Platelet Aggregating Factor,Platelet Aggregation Enhancing Factor,Platelet-Activating Substance,Thrombocyte Aggregating Activity,1 Alkyl 2 acetyl sn glycerophosphocholine,Aggregating Factor, Platelet,Factor, Platelet Activating,PAF Acether,Platelet Activating Substance
D011464	Epoprostenol	A prostaglandin that is a powerful vasodilator and inhibits platelet aggregation. It is biosynthesized enzymatically from PROSTAGLANDIN ENDOPEROXIDES in human vascular tissue. The sodium salt has been also used to treat primary pulmonary hypertension (HYPERTENSION, PULMONARY).	Prostacyclin,Prostaglandin I2,Epoprostanol,Epoprostenol Sodium,Epoprostenol Sodium Salt, (5Z,9alpha,11alpha,13E,15S)-Isomer,Flolan,Prostaglandin I(2),Veletri
D011693	Purpura	Purplish or brownish red discoloration, easily visible through the epidermis, caused by hemorrhage into the tissues. When the size of the discolorization is >2-3 cm it is generally called Ecchymoses (ECCHYMOSIS).	Petechiae,Purpuras
D011697	Purpura, Thrombotic Thrombocytopenic	An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. Mutations in the ADAMTS13 PROTEIN gene have been identified in familial cases.	Moschkowitz Disease,Purpura, Thrombotic Thrombopenic,Thrombotic Thrombocytopenic Purpura, Congenital,Thrombotic Thrombocytopenic Purpura, Familial,Congenital Thrombotic Thrombocytopenic Purpura,Familial Thrombotic Thrombocytopenia Purpura,Familial Thrombotic Thrombocytopenic Purpura,Microangiopathic Hemolytic Anemia, Congenital,Moschcowitz Disease,Schulman-Upshaw Syndrome,Thrombotic Microangiopathy, Familial,Thrombotic Thrombocytopenic Purpura,Upshaw Factor, Deficiency of,Upshaw-Schulman Syndrome,Familial Thrombotic Microangiopathy,Microangiopathy, Familial Thrombotic,Schulman Upshaw Syndrome,Thrombocytopenic Purpura, Thrombotic,Thrombopenic Purpura, Thrombotic,Thrombotic Thrombopenic Purpura,Upshaw Schulman Syndrome
D001779	Blood Coagulation Factors	Endogenous substances, usually proteins, that are involved in the blood coagulation process.	Clotting Factor,Coagulation Factors,Blood Coagulation Factor,Clotting Factors,Coagulation Factor,Coagulation Factor, Blood,Coagulation Factors, Blood,Factor, Coagulation,Factors, Coagulation,Factor, Blood Coagulation,Factor, Clotting,Factors, Blood Coagulation,Factors, Clotting
D001792	Blood Platelets	Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation.	Platelets,Thrombocytes,Blood Platelet,Platelet,Platelet, Blood,Platelets, Blood,Thrombocyte
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children