Thrombotic thrombocytopenic purpura unresponsive to plasma infusion and plasma exchange, but responsive to splenectomy.
A 60-yr-old female presented with typical thrombotic thrombocytopenic purpura (TTP). She remained in coma with frequent seizures for 1 wk, with persisting severe thrombocytopenia and microangiopathic haemolytic anaemia, despite treatment with prednisolone, plasma exchange, fresh frozen plasma, sulphinpyrazone and dipyridamole. Splenectomy induced haematological improvement within 1 d, there was cessation of fitting after 2 d, and full neurological recovery ensued over 3 wk. Laboratory studies did not reveal the presence of a platelet-aggregating factor (PAF), stated to be present in some two-thirds of cases. While plasma exchange and plasma infusion are beneficial in many cases, splenectomy appears still to be of value in unresponsive disease.
D001803:Blood Transfusion; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008875:Middle Aged; D010949:Plasma; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000473:pathology / Q000628:therapy*; D013156:Splenectomy; D013997:Time Factors