We presented two patients whoinitially presented with thrombotic microangiopathy and were later diagnosedwith TTP upon demonstration of the deficiency in ADAMTS-13 activity.
📌 3 Key Takeaways
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Objective & Hypothesis:Thrombotic thrombocytopenic purpura (TTP) is a rare multisystemdisorder characterized by single or recurrent episodes of thrombocytopenia,microangiopathic hemolytic anemia and widespread microvascular thrombosis,which causes significant morbidity and mortality unless promptly recognizedand treated.
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Key Findings & Data:The underlying pathogenesis is a defect in von Willebrandfactor (vWF) cleaving protease, called “A Disintegrin and Metalloproteinasewith Thrombospondin Type 1 Repeats 13 (ADAMTS-13)”.
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Conclusion & Impact:We presented two patients whoinitially presented with thrombotic microangiopathy and were later diagnosedwith TTP upon demonstration of the deficiency in ADAMTS-13 activity.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters
Özet
Thrombotic thrombocytopenic purpura (TTP) is a rare multisystemdisorder characterized by single or recurrent episodes of thrombocytopenia,microangiopathic hemolytic anemia and widespread microvascular thrombosis,which causes significant morbidity and mortality unless promptly recognizedand treated. The underlying pathogenesis is a defect in von Willebrandfactor (vWF) cleaving protease, called “A Disintegrin and Metalloproteinasewith Thrombospondin Type 1 Repeats 13 (ADAMTS-13)”. There are 2forms: congenital TTP (ADAMTS-13 gene mutations) and acquired TTP(autoantibodies and ADAMTS-13 deficiency). We presented two patients whoinitially presented with thrombotic microangiopathy and were later diagnosedwith TTP upon demonstration of the deficiency in ADAMTS-13 activity.
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Cite This Article
KÖKER, O., YILDIRMAK, Z. Y., GENÇ, D. B., KILIÇASLAN, Ö. (2019). Thrombotic thrombocytopenic purpura as a rare cause of anemia with thrombocytopenia in childhood: report of 2 cases. Turkish Journal of Pediatrics, Cilt: 61 - Sayı: 3, 418–423. https://ajindex.com/articles/411996
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