A 35-year-old male patient with long lasting HIV infection presented with c
onfusion, haemolytic anaemia and thrombocytopenia. After a stable course ov
er 7 days he suddenly developed coma and cardiogenic shock and died. Autops
y revealed thrombotic thrombocytopenic purpura with fibrin-rich deposits in
the microvasculature of the brain, heart and pancreas. The pathogenesis an
d differential diagnosis of this fulminant disease are discussed.