Thrombotic thrombocytopenic purpura: A treatable cause of childhood encephalopathy☆,☆☆,★
Section snippets
Patient 1
A 12-year-old boy was admitted with fever, malaise, arthralgia, and myalgia of 1 week's duration. There was a 2-month history of intermittent pruritic rash. The medical and family history were unremarkable. On admission the temperature was 37.9° C, respiratory rate 24 breaths/min, heart rate 96 beats/min, and blood pressure 125/75 mm Hg. He had a maculopapular eruption on his trunk and limbs, generalized tender lymphadenopathy, and hepatomegaly. There was a painful effusion of his right knee,
DISCUSSION
The clinical syndrome of TTP, first described by Moschcowitz 7 in 1924, is a pentad of fever, MAHA, thrombocytopenia, neurologic abnormalities, and renal dysfunction. In 1955, Gasser et al. 8 described HUS, a condition with presentation in childhood, with intravascular hemolysis, thrombocytopenia, renal failure, and occasionally neurologic or psychiatric symptoms. It is probable that these two disorders represent different clinical manifestations of the same pathologic process, namely platelet
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Cited by (14)
Long-term remission of recurrent thrombotic thrombocytopenic purpura (TTP) after Rituximab in children and young adults
2015, Pediatric Blood and CancerThrombotic thrombocytopenic purpura in pediatric patients
2012, Chinese Journal of Contemporary PediatricsThrombotic thrombocytopenic purpura
2011, Pediatric Emergency CareCase of TTP with cerebral infarct secondary to platelet transfusion
2011, Indian Journal of PediatricsPlasma filtration on mediators of thrombotic microangiopathy: An in vitro study
2007, International Journal of Artificial OrgansThrombotic Thrombocytopenic Purpura
2004, Indian Pediatrics
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From the Departments of Pediatrics and Pathology, University of British Columbia, British Columbia's Children's Hospital, Vancouver, Canada.
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Reprint requests: L. D. Wadsworth, MBChB, Program Director, Department of Hematopathology, British Columbia's Children's Hospital, 4480 Oak St., Vancouver, BC V6H 3V4, Canada.
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