Elsevier

The Journal of Pediatrics

Volume 130, Issue 2, February 1997, Pages 313-316
The Journal of Pediatrics

Thrombotic thrombocytopenic purpura: A treatable cause of childhood encephalopathy,☆☆,

https://doi.org/10.1016/S0022-3476(97)70362-7Get rights and content

Abstract

We describe two patients less than 13 years of age with thrombotic thrombocytopenic purpura, a rare disorder in childhood. Both children were treated with plasma exchange therapy, which resulted in a rapid resolution of symptoms. This disorder is a cause of childhood encephalopathy, which can be treated effectively with plasma exchange.(J Pediatr 1997;130:313-6)

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

References (14)

There are more references available in the full text version of this article.

From the Departments of Pediatrics and Pathology, University of British Columbia, British Columbia's Children's Hospital, Vancouver, Canada.

☆☆

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.

0022-3476/97/$5.00 + 0 9/22/77544

View full text