Abstract
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.
MeSH terms
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ADAM Proteins
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ADAMTS13 Protein
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Adolescent
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Autoantibodies / blood
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Autoantibodies / immunology
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Autoimmune Diseases / epidemiology
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Autoimmune Diseases / immunology
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Blood Component Transfusion
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Cerebral Hemorrhage / etiology
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Child
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Child, Preschool
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Consciousness Disorders / etiology
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Diabetes Mellitus, Type 1 / complications
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Female
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Humans
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Infant
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Kidney Tubular Necrosis, Acute / complications
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Male
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Metalloendopeptidases / deficiency
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Metalloendopeptidases / genetics
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Metalloendopeptidases / immunology
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Paresis / etiology
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Plasma
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Plasma Exchange
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Purpura, Thrombotic Thrombocytopenic / enzymology
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Purpura, Thrombotic Thrombocytopenic / epidemiology*
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Purpura, Thrombotic Thrombocytopenic / etiology
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Purpura, Thrombotic Thrombocytopenic / pathology
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Purpura, Thrombotic Thrombocytopenic / therapy
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Texas / epidemiology
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Thyroiditis, Autoimmune / complications
Substances
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Autoantibodies
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ADAM Proteins
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Metalloendopeptidases
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ADAMTS13 Protein
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ADAMTS13 protein, human