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

Autor: Elise Schapkaitz, Tanya Marié Schickerling
Rok vydání: 2018
Předmět:
Zdroj: Laboratory Medicine.
ISSN: 1943-7730
0007-5027
DOI: 10.1093/labmed/lmy015
Popis: 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.
Databáze: OpenAIRE