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Lab Med. 2018 Jul 5;49(3):268-271. doi: 10.1093/labmed/lmy015.

The Diagnostic Challenge of Acquired Thrombotic Thrombocytopenic Purpura in Children: Case Report and Review of the Literature.

Author information

1
Department of Hematology and Molecular Medicine, Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa.
2
Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
3
Division of Pediatric Hematology and Oncology, Department of Pediatrics and Child Health, Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa.

Abstract

Acquired thrombotic thrombocytopenic purpura (TTP) is a rare disease in children. Despite advances in diagnosis and treatment, acquired TTP remains a challenging disease due to the lack of a simple diagnostic test and the variable response to plasma exchange. Herein, we describe a case of a 5-year-old of black ethnicity boy who presented with a sudden onset of fatigue and body aches. Laboratory investigations revealed a thrombotic microangiopathic manifestation. TTP was diagnosed, and plasma exchange and corticosteroids were initiated, with an excellent response. Subsequently, reduced disintegrin and metalloprotease with thrombospodin-2-like repeats (ADAMTS-13) activity and human immunodeficiency virus (HIV) were confirmed. Antiretroviral treatment was started as long-term management. At last follow-up, he continues in stable remission.

PMID:
29659973
DOI:
10.1093/labmed/lmy015
[Indexed for MEDLINE]

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