H. Ferro et al., Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Three cases with simultaneous presentation., MEDICINA, 59(6), 1999, pp. 739-742
Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome characteri
zed by microangiopathic hemolytic anemia, thrombocytopenia, fever, neurolog
ical symptoms, and renal involvement. The relationship of TTP to systemic l
upus erythematosus (SLE) has been recognized in the medical literature sinc
e 1939. The differential diagnosis is difficult because both diseases have
similar clinical features. The mainstay for recognizing TTP in the context
of active SLE is the presence of helmet red cells, marked reticulocytosis,
and negative direct Coomb's test. We report three female patients with simu
ltaneous presentation of TTP and SLE. We suggest combined treatment with im
munosuppressive therapy and plasma exchange using fresh frozen plasma.