Introduction: Thrombotic thrombocytopenic purpura (TTP) is a hematological disease resulting from the ADAMTS 13 plasmatic protein deficit. It can be congenital or sporadic, and is usually autoimmune. Pathological platelet adhesion occurs, leading to microthrombi in capillary and arterial circulation, microangiopathic anemia and ischemia. The clinical picture includes thrombocytopenia, renal dysfunction, fluctuating neurological symptoms, microangiopathic hemolytic anemia, and fever. Methods: Case report of a 51-year-old male hypertensive patient, diagnosed with idiopathic thrombocytopenic purpura (ITP) 10 years ago and submitted to splenectomy 5 years ago, who developed acute cholecystitis. He underwent urgent colecistectomy, and on the fourth postoperative day presented sudden space and time disorientation, transcortical motor aphasia and right faciobrachial paresis, with ipsilateral Babinski and Hoffman signs. Results: Brain CT showed left frontoparietal hypodensity. During hospitalization, there was worsening of renal function, increased LDH, and thrombocytopenia. Hematoscopy identified signs of intravascular hemolysis (erythrocyte fragmentation, reticulocytosis, helmet erythrocytes). Direct Coombs was negative. There was no history of heparin use. TTP was diagnosed, and fresh frozen plasma and prednisone 1mg/kg were prescribed. There was resolution of thrombotic microangiopathy, with subsequent increase of platelet levels, decreased LDH and improved hematoscopy. Conclusions: This case illustrates a rare cause of stroke and an unusual association of two hematological conditions: ITP and TTP. The treatment of TTP consists of replacement of deficient ADAMTS13 protein through plasmapheresis or fresh frozen plasma. The use of immunosuppressants is also associated, initially with glucocorticoids, followed by rituximab or splenectomy in order to prevent recurrences.
Factor XIII deficiency leading to preseptal haematoma post-strabismus surgery
