Publication:

A case report of thrombotic thrombocytopenic purpura with positive ANA activity

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder, characterized by: consumptive thrombocytopenia, microangiopathic hemolytic anemia with the presence of schistocytes, renal impairment, neurological dysfunction and fever. A 12-years-old girl presented with TTP and had ADAMTS13 activity deficiency along with positive ANA activity. The child was planned for plasmapheresis but developed refractory seizures and pulmonary bleeding and succumbed to death. TTP with ANA positivity has a very fulminant course. ⁠Pulmonary involvement can be asymptomatic but has a poor prognosis with rapid clinical deterioration. A high index of suspicion is of utmost importance in low to middle-income countries settings as the facility of plasmapheresis is available at very few centers