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Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature.

Authors :
Alias H
Yong WL
Muttlib FAA
Koo HW
Loh CK
Lau SCD
Alauddin H
Azma RZ
Source :
Journal of medical case reports [J Med Case Rep] 2018 Sep 17; Vol. 12 (1), pp. 276. Date of Electronic Publication: 2018 Sep 17.
Publication Year :
2018

Abstract

Background: Acquired thrombotic thrombocytopenia purpura is very rarely encountered in children. It is often misdiagnosed initially when the condition is not inherited.<br />Case Presentation: We describe a 3-year-old Malay boy who presented with simple febrile seizure and had no neurological deficit, however, he was found to have microangiopathic hemolytic anemia, thrombocytopenia, and elevated serum lactate dehydrogenase. An ADAMTS13 assay results showed zero activities (0%), and markedly high level of ADAMTS13 inhibitor (93.15 U/mL) confirming the diagnosis of secondary thrombotic thrombocytopenia purpura. He received fresh frozen plasma infusions for 3 days and subsequently his platelet levels normalized. Serial ADAMTS13 assay results showed improvement. He was also given a short course of prednisolone after which the ADAMTS13 activity normalized (> 114%) at the end of prednisolone course.<br />Conclusions: At presentation, acquired thrombotic thrombocytopenia purpura in a very young child is commonly misdiagnosed as other conditions like idiopathic thrombocytopenic purpura, Evans syndrome, atypical hemolytic-uremic syndrome, or malignancy. ADAMTS13 assay should be performed early when thrombotic thrombocytopenia purpura is suspected as this condition is associated with dire consequences.

Details

Language :
English
ISSN :
1752-1947
Volume :
12
Issue :
1
Database :
MEDLINE
Journal :
Journal of medical case reports
Publication Type :
Academic Journal
Accession number :
30223886
Full Text :
https://doi.org/10.1186/s13256-018-1806-9