Meeting abstract
https://doi.org/10.26800/LV-145-supl8-15
A rare case of hereditary thrombotic thrombocytopenic purpura in a toddler
Ozana Jakšić
Jana Jelčić
Maša Davidović
Abstract
Hereditary thrombotic thrombocytopenic purpura (hTTP) is a thrombotic microangiopathy caused by pathogenic variants in the ADAMTS13 gene resulting in highly reduced activity of von Willebrand factor-cleaving metalloprotease. hTTP is characterized by systemic for-mation of platelet-rich thrombi in microvasculature resulting in organ ischemia, throm-bocytopenia, and microangiopathic hemolytic anemia.
Keywords
ADAMTS13 protease, pediatrics, thrombotic thrombocytopenic purpura
Hrčak ID:
314111
URI
Publication date:
22.2.2024.
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