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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

BACKGROUND: Acquired thrombotic thrombocytopenia purpura is very rarely encountered in children. It is often misdiagnosed initially when the condition is not inherited. CASE PRESENTATION: We describe a 3-year-old Malay boy who presented with simple febrile seizure and had no neurological deficit, ho...

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Autores principales: Alias, Hamidah, Yong, Woon Lee, Muttlib, Farah Azima Abdul, Koo, Ho Wai, Loh, C-Khai, Lau, Sie Chong Doris, Alauddin, Hafiza, Azma, Raja Zahratul
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6142628/
https://www.ncbi.nlm.nih.gov/pubmed/30223886
http://dx.doi.org/10.1186/s13256-018-1806-9
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author Alias, Hamidah
Yong, Woon Lee
Muttlib, Farah Azima Abdul
Koo, Ho Wai
Loh, C-Khai
Lau, Sie Chong Doris
Alauddin, Hafiza
Azma, Raja Zahratul
author_facet Alias, Hamidah
Yong, Woon Lee
Muttlib, Farah Azima Abdul
Koo, Ho Wai
Loh, C-Khai
Lau, Sie Chong Doris
Alauddin, Hafiza
Azma, Raja Zahratul
author_sort Alias, Hamidah
collection PubMed
description BACKGROUND: Acquired thrombotic thrombocytopenia purpura is very rarely encountered in children. It is often misdiagnosed initially when the condition is not inherited. 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. 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.
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spelling pubmed-61426282018-09-20 Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature Alias, Hamidah Yong, Woon Lee Muttlib, Farah Azima Abdul Koo, Ho Wai Loh, C-Khai Lau, Sie Chong Doris Alauddin, Hafiza Azma, Raja Zahratul J Med Case Rep Case Report BACKGROUND: Acquired thrombotic thrombocytopenia purpura is very rarely encountered in children. It is often misdiagnosed initially when the condition is not inherited. 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. 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. BioMed Central 2018-09-17 /pmc/articles/PMC6142628/ /pubmed/30223886 http://dx.doi.org/10.1186/s13256-018-1806-9 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Alias, Hamidah
Yong, Woon Lee
Muttlib, Farah Azima Abdul
Koo, Ho Wai
Loh, C-Khai
Lau, Sie Chong Doris
Alauddin, Hafiza
Azma, Raja Zahratul
Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature
title Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature
title_full Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature
title_fullStr Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature
title_full_unstemmed Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature
title_short Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature
title_sort acquired thrombotic thrombocytopenia purpura associated with severe adamts13 deficiency in a 3-year-old boy: a case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6142628/
https://www.ncbi.nlm.nih.gov/pubmed/30223886
http://dx.doi.org/10.1186/s13256-018-1806-9
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