Thrombotic thrombocytopenic purpura. An extended editorial.

A hypothesis for the pathogenesis of TTP is developed, centered on the views and findings of Kwaan (Fig. 8). By a series of deductions, inferences, and extensions, an attempt has been made to define considerably more restrictively than heretofore a TTP paradigm of clinical (Tables 1,2,3) and laboratory (Table 4) features. Revisions may be required in the future as increase of knowledge indicates. By similar methods rational, exigent therapeutic measures are selected (Table 5). The innovative therapeutic use of plasma transfusion, as proposed by Byrnes and Lian and their co-workers, is compatible with the paradigm in all respects. The paradigm discriminates for authentic cases of TTP among spurious if similar entities. Such sequestration will constitute a homogeneous universe of case material appropriate for study. The prospective use of the TTP paradigm defined in this papd therapeutic protocol, jointly developed, may accelerate our knowledge and improve our effectiveness in this lethal, if uncommon, disease [49,76,111,170].