Thrombotic thrombocytopenia purpura in a Nigerian girl
Authors: Kaladada I. Korubo, MB.BS, FMCPath
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Thrombotic thrombocytopenic purpura is a rare congenital or acquired disorder either due to mutations in the ADAMTS13 gene or development of antibodies to the ADAMTS13 protein. TTP causes a life-threatening form of thrombotic microangiopathy that is characterized by thrombocytopenia, microangioapathic haemolytic anaemia, fever, neurological symptoms and renal impairment. ADAMTS13 functions to cleave large von Willebrand factor multimers and in its absence there is unregulated VWF-dependent platelet thrombosis. A nineteen year old female presented with a history of menorrhagia, petechiae and purpural haemorrhages, painful right jaw, anaemia of 8.1g/dL and thrombocytopenia of 31 X 109/L. Her blood film showed numerous schistocytes with thrombocytopenia on film. Bone marrow aspirate revealed increased megakaryocytic and erythroid activity. The uric acid and lactate dehydrogenase parameters were elevated but her renal functions and other investigations were essentially normal. At presentation she was initially commenced on high dose prednisolone, however on review of the peripheral blood schistocytes and thrombocytopenia a provisional diagnosis of thrombotic thrombocytopenia was made and ADAMTS13 was done which revealed low ADAMTS13 levels. This is a case report to document this rare disorder for the first time in our environment which was successfully treated without the use of plasma exchange, plasma infusion or blood transfusion but rather with high dose prednisolone.