Christopher P. Coppola, Alfred P. Kennedy, Jr. and Ronald J. Scorpio (eds.)Pediatric Surgery2014Diagnosis and Treatment10.1007/978-3-319-04340-1_54
© Springer International Publishing Switzerland 2014
Thrombotic Thrombocytopenic Purpura
(1)
Department of Pediatric Hematology/Oncology, Janet Weis Children’s Hospital, 100 N. Academy Av. MC 13-20, Danville, PA 17822, USA
Abstract
Thrombotic Thrombocytopenic Purpura (TTP) is a syndrome marked by waxing and waning episodes of organ and tissue ischemia secondary to reversible platelet aggregation in the microvasculature. It often presents with thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, fever and renal abnormalities. Without treatment the mortality rate may reach 90 %. TTP is usually acquired although inherited chronically relapsing forms also occur.
Thrombotic Thrombocytopenic Purpura (TTP) is a syndrome marked by waxing and waning episodes of organ and tissue ischemia secondary to reversible platelet aggregation in the microvasculature. It often presents with thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, fever and renal abnormalities. Without treatment the mortality rate may reach 90 %. TTP is usually acquired although inherited chronically relapsing forms also occur.
1.
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Pathophysiology:
(a)
Characterized by thrombi containing aggregated platelets with little inflammatory response.
(b)
Lesions usually involve small vessels (arterioles and capillaries).
(c)
Brain, abdominal organs and the heart are most commonly involved.
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