Case series of thrombotic thrombocytopenic purpura in children and adolescents.
Thrombotic thrombocytopenic purpura (TTP) is a well-described entity in adults but is rarely observed in children. The authors describe a series of seven children with suspected acquired TTP. Clinical findings included petechiae, purpura, or jaundice ( 6), central nervous system events ( 5), fever ( 3), diarrhea ( 3), renal insufficiency ( 2), and hematuria ( 2). Significant central nervous system events included cerebral vascular accidents ( 2), altered mental status ( 2), seizures ( 1), and hemiparesis ( 1). Patients were treated with daily plasma infusions (1/7) or plasma exchange (5/7). Response was prompt, although relapses were frequent. Decreased vWF-protease activity was found in four of five cases and vWF-protease inhibitors were found in three of five cases. Although rare, TTP is a life-threatening illness that does occur in children and should be considered in the differential diagnosis of thrombocytopenia with hemolytic anemia.
Horton
Terzah M
TM
Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas 77030, USA.
Stone
Jeffrey D
JD
Yee
Donald
D
Dreyer
ZoAnn
Z
Moake
Joel L
JL
Mahoney
Donald H
DH
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000293:Adolescent; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001327:Autoimmune Diseases / Q000453:epidemiology / Q000276:immunology; D016913:Blood Component Transfusion; D002543:Cerebral Hemorrhage / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003244:Consciousness Disorders / Q000209:etiology; D003922:Diabetes Mellitus, Type 1 / Q000150:complications; D005260:Female; D006801:Humans; D007223:Infant; D007683:Kidney Tubular Necrosis, Acute / Q000150:complications; D008297:Male; D008666:Metalloendopeptidases / Q000172:deficiency / Q000235:genetics / Q000276:immunology; D010291:Paresis / Q000209:etiology; D010949:Plasma; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000201:enzymology / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology / Q000628:therapy; D013781:Texas / Q000453:epidemiology; D013967:Thyroiditis, Autoimmune / Q000150:complications
D001323:Autoantibodies; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human