Thrombotic thrombocytopenic purpura. An immune-complexes disease?
Celada, A.; Laso, F.J.; Perrin, L.H.
Sangre 24(5): 593-599
1979
ISSN/ISBN: 0036-4355 PMID: 161419 Document Number: 153135
A 31 yr old female with thrombotic thrombocytopenic purpura was presented. Treatment with heparin and anti-aggregating drugs induced no improvement at all. Steroid therapy was followed by complete remission. The presence of immune complexes was investigated during the course of the disease by 2 different methods (Clq [q fragment of complement component 1] binding test and conglutinin binding test). Total hemolytic complement levels and the fragments of the classic or alternative pathways, Clq, C4, C3, factor B and C3d (a split product of C3) were within the normal range. The assessment of antinuclear antibodies and anti-DNA native or denatured was irrelevant. No perivascular immunoglobulin or C depositions were found in a skin biopsy. In spite of the great number of indirect arguments favoring the existence of circulating immune complexes in thrombotic thrombocytopenic purpura, the present case, as well as 3 other reported in the literature, fail to demonstrate a possible etiopathogenetic role played by immune complexes in this disease.