[Systemic lupus erythematosus and thrombotic thrombocytopenic purpura].

JAPI • VOL. 51 • JULY 2003 smear shows red cell fragments, schistocytes, and helmet cells suggestive of hemolysis. Other features suggestive of MAHA are reticulocytosis, indirect hyperbilirubinina, hemoglobinuria, nucleated red cells and increased LDH. Coagulation parameters are normal except increased FDP. Histopathology shows hyaline thrombi and microthrombi in arterioles and capillaries. Mortality is 50-80%. Various treatment modalities used are steroids, antiplatelet drugs, plasma infusion splenectomy and plasmapharesis. The association of TTP and SLE is highly debated. It has been recognized in the medical literature since 1939.1 Jonsson OG and Fink CW2 described a 10 year old who presented with TTP and shortly thereafter developed SLE. This is the first report of SLE presenting TTP. Fourty cases of TTP in association with SLE are reported in world literature.3 Review of the literature revealed that TTP might occur in the setting of either active or inactive SLE. TTP may be caused by many factors, one of which is SLE. It can present as a terminal event in SLE or SLE has also been shown to initially present several years after TTP. Pediatric TTP with high-grade proteinuria is a predictor of SLE. Etiology of TTP is elusive. It is unknown if the endothelial damage represents the first lesion or if the platelet hyperaggregability precedes the vascular injury. Certain autoimmune mecanisms, platelet abnormalities, fibrinolytic disorders may be shared with SLE and provide the basis for their association.3 Also a case of primary APLA with TTP has been described in pregnancy. Relationship between these two is controversial. Montecucco et al 4 measured APLA in nine patients with active TTP, eight had primary TTP and one had secondary to SLE. No patient showed circulating LA or false positive test for syphilis. Autoantibodies to CD36, a platelet glycoprotein have been found in patients with TTP and in those with lupus like anticoagulant with thrombotic complications.5 Koyama T et al described immunological changes in cases of TTP.6 One case that was diagnosed TTP complicated with SLE showed some serological abnormalities of LE test, anti-sterptokinase anti-DNA antibodies, and platelet bound IgG and serum level of IgG by renal biopsy with little changes in complement levels. Platelet associated IgG (PA IgG) was positive in four of six cases and this seemed to be the only abnormality associated with remission and relapse of TTP. Survival in SLE associated TTP correlated with the use of plasma therapy (infusion/plasmapharesis) rather than with the activity of the underlying autoimmune disease. Patients treated with plasmapharesis/infusion had a lower mortality rate at 25% as compared with 57% mortality in those who were not treated with plasmapharesis. Addition of cyclophosphamide to treatment with plasmapharesis and steroids was beneficial. Management requires timely diagnosis and aggressive treatment by therapeutic plasma exchange. SS Suralkar*, LS Bichile**, AS Sonawale*, AG Rajyadhyaksha***, Guruprasad# *Lecturer; **Professor and Head; ***Associate Professor; Systemic Lupus Erythematosus with Thrombotic Thrombocytopenic Purpura