THROMBOTIC THROMBOCYTOPENIC PURPURA AND H EMOLYTIC-UREMIC SYNDROME INADULT - A RETROSPECTIVE STUDY OF 27 PATIENTS

Citation
N. Hamdini et al., THROMBOTIC THROMBOCYTOPENIC PURPURA AND H EMOLYTIC-UREMIC SYNDROME INADULT - A RETROSPECTIVE STUDY OF 27 PATIENTS, Annales de medecine interne, 148(5), 1997, pp. 346-355
Citations number
52
Categorie Soggetti
Medicine, General & Internal
Journal title
ISSN journal
0003410X
Volume
148
Issue
5
Year of publication
1997
Pages
346 - 355
Database
ISI
SICI code
0003-410X(1997)148:5<346:TTPAHE>2.0.ZU;2-5
Abstract
We report a serie of 27 patients included on the basis of either throm botic microangiopathy (TMA) at renal histology (13 cases) or, in the a bsence of histology, non-immunological hemolytic anemia with schizocyt es and thrombopenia (14 cas). The etiopathogenic treatment consisted i n the administration of antiagregating agents (in all patients except 3 of group I because of the severity of thrombopenic). corticosteroids (1 case), intravenous immunoglobulins[ins (2 cases) fresh frozen plas ma (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: p artial renal recovery; IV:complete renal recovery. Comments and conclu sion a/Patients with neurological complications have poor prognosis in spite of minor renal involvement and use of PE whose indication is va lidated in these cases. b/When renal involvement predominates, acceler ated hypertension is linked to arteriolar or mixte type of TMA, expose s to an increased risk of hemorrhagic complications of the renal biops y (4 out of 5) which questions the usefulness of such biopsy (group II ). c/TMA may precede cancer. It has per se a favorable outcome even wh en metastases are already present, warranting agressive treatment.