Thrombotic thrombocytopenic purpura as a rare cause of anemia with thrombocytopenia in childhood: report of 2 cases

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.