Thrombotic microangiopathy: a case report and review of the literature

Thrombotic microangiopathy most likely represents a spectrum of diseases consisting of multiple etiologies that has a final common pathway of multiorgan microvascular thrombosis. The variable responses to several different modes of therapy would suggest that more than one pathogenetic mechanism is involved. Untreated, it has been associated with very high morbidity and mortality rates. A poor understanding of the basic disease process has prevented specific treatment modalities, although early diagnosis and availability of dialysis and blood product transfusion services remain crucial. Several modes of therapy have been used to date, with plasma exchange being the most effective method studied and shown to improve survival. On the basis of current knowledge, this form of treatment should be instituted promptly in severe cases. Anecdotal reports of recovery with vincristine or IgG alone or with the use of IgG after the apparent failure of plasma therapy appear promising and deserve further investigation as initial therapeutic measures used in thrombotic microangiopathy. Although the majority of patients recover with normal renal function, those with severe thrombotic microangiopathy may heal through sclerosis with residual hypertension and chronic renal impairment requiring continual medical therapy.