[Thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome in adults. Apropos of 27 cases]

Abstract

We report a series of 27 patients included on the basis of either thrombotic microangiopathy (TMA) at renal histology (13 cases) or, in the absence of histology, non-immunological hemolytic anemia with schizocytes and thrombopenia (14 cas). The etiopathogenic treatment consisted in the administration of antiagregating agents (in all patients except 3 of group I because of the severity of thrombopenic), corticosteroids (1 case), intravenous immunoglobulins (2 cases) fresh frozen plasma (FFP) without plasma exchange (PE) in 7 cases and PE with FFP in 13 patients. According to the 6 months outcome, 4 groups were considered I: death due to neurological damage; II: chronic hemodialysis; III: partial renal recovery; IV: complete renal recovery.

Comments and conclusions: a/Patients with neurological complications have poor prognosis in spite of minor renal involvement and use of PE whose indication is validated in these cases. b/When renal involvement predominates, accelerated hypertension is linked to arteriolar or mixte type of TMA, exposes to an increased risk of hemorrhagic complications of the renal biopsy (4 out fo 5) which questions the usefulness of such biopsy (group II). c/TMA may precede cancer. It has per se a favorable outcome even when metastases are already present, warranting aggressive treatment.