Document Detail


Acquired, noncongenital thrombotic thrombocytopenic purpura in children and adolescents: clinical management and the use of ADAMTS 13 assays.
MedLine Citation:
PMID:  20083997     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Thrombotic thrombocytopenic purpura (TTP) in children is rare and is often thought to be due to congenital ADAMTS13 deficiency. We report seven new cases of noncongenital TTP in children and adolescents and perform a review of the literature where ADAMTS13 assays have been performed in paediatric acquired TTP. All new cases were female and the median age was 13 years. Presenting clinical features included bruising/petechiae or bleeding, fever, neurological, and renal impairment. Median Hb and platelet counts on admission were 66 g/l and 10 x 10(9)/l respectively. Two cases had raised Troponin T levels and one had an abnormal ECG. All cases had ADAMTS13 activity less than 5% and an inhibitor to ADAMTS13. The median number of plasma exchange to remission was 22.5. Six cases received rituximab. Three achieved normal ADAMTS13 activity and remain in remission. Two had persistently low ADAMTS13 activity with high anti-ADAMTS13 IgG levels and one of these relapsed. One had moderately reduced levels of ADAMTS13 in remission with no inhibitor, however, a fall in ADAMTS13 activity and increase in anti-ADAMTS13 IgG heralded clinical relapse. The literature review identified 12 acquired cases showing low ADAMTS13 activity and inhibition of ADAMTS13 (in 95%). In children and adolescents TTP may be due to acquired deficiency of ADAMTS13, associated with an inhibitor/Anti-ADAMTS13 IgG antibodies. Treatment of acquired disease requires PEX and usually immunosuppressive treatment. Rituximab appears to be an effective adjunctive treatment modality.
Authors:
Vickie McDonald; Ri Liesner; John Grainger; Michael Gattens; Samuel J Machin; Marie Scully
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis     Volume:  21     ISSN:  1473-5733     ISO Abbreviation:  Blood Coagul. Fibrinolysis     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-03-30     Completed Date:  2010-06-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9102551     Medline TA:  Blood Coagul Fibrinolysis     Country:  England    
Other Details:
Languages:  eng     Pagination:  245-50     Citation Subset:  IM    
Affiliation:
Haemostasis Research Unit, Department of Haematology, University College London, UK. v.mcdonald@ucl.ac.uk
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MeSH Terms
Descriptor/Qualifier:
ADAM Proteins / metabolism*
Adolescent
Antibodies, Monoclonal / therapeutic use
Child
Female
Humans
Immunologic Factors / therapeutic use
Plasma Exchange
Purpura, Thrombotic Thrombocytopenic / diagnosis*,  drug therapy,  therapy*
Chemical
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Immunologic Factors; 0/rituximab; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human

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