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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.

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


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.

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