The Turkish Journal of Pediatrics 2019 , Vol 61 , Num 3
Thrombotic thrombocytopenic purpura as a rare cause of anemia with thrombocytopenia in childhood: report of 2 cases
Oya Köker 1 ,Zeynep Yıldız Yıldırmak 2 ,Dildar Bahar Genç 2 ,Önder Kılıçaslan 1
1 Department of Pediatrics, Şişli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey
2 Department of Pediatric Hematology and Oncology, Şişli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey
DOI : 10.24953/turkjped.2019.03.015 Köker O, Yıldırmak ZY, Genç DB, Kılıçaslan Ö. Thrombotic thrombocytopenic purpura as a rare cause of anemia with thrombocytopenia in childhood: report of 2 cases. Turk J Pediatr 2019; 61: 418-423.

Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem disorder characterized by single or recurrent episodes of thrombocytopenia, microangiopathic hemolytic anemia and widespread microvascular thrombosis, which causes significant morbidity and mortality unless promptly recognized and treated. The underlying pathogenesis is a defect in von Willebrand factor (vWF) cleaving protease, called `A Disintegrin and Metalloproteinase with Thrombospondin Type 1 Repeats 13 (ADAMTS-13)`. There are 2 forms: congenital TTP (ADAMTS-13 gene mutations) and acquired TTP (autoantibodies and ADAMTS-13 deficiency). We presented two patients who initially presented with thrombotic microangiopathy and were later diagnosed with TTP upon demonstration of the deficiency in ADAMTS-13 activity. Keywords : ADAMTS-13, microangiopathy, Thrombotic thrombocytopenic purpura

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