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
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.