Treatment outcomes in patients with adult thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
BACKGROUND
Plasma treatment has improved the outcomes in adults with thrombotic thrombocytopenic purpura (TTP)-hemolytic uremic syndrome (HUS). We reviewed our experience in treating unselected patients to determine the clinical outcomes and to evaluate the treatments given in addition to plasma.


METHODS
A chart review of all cases of TTP and HUS in adults treated at the Toronto (Ontario) Hospital, the largest treatment center for adults with TTP-HUS in the province of Ontario, was conducted.


RESULTS
Sixty-seven episodes of TTP-HUS in 52 consecutive adult patients were treated during a 12-year period. Plasma was the primary form of therapy, and most patients received plasma exchange. A complete hematologic remission was achieved in 65 of 67 episodes; however, two patients in remission were brain-dead. The time to complete remission varied from 3 to 58 days (median, 13 days). The death rate during the acute illness was 8%. Long-term sequelae included relapses, persisting renal impairment, hepatitis, and transfusion-associated acquired immunodeficiency syndrome. Relapses occurred in 21% of patients during a median follow-up of 1.1 years (range, 0.1 to 18 years). Analyses of the treatment given in addition to plasma did not demonstrate a significant benefit in terms of reducing the illness duration, mortality, or long-term sequelae.


CONCLUSIONS
While most patients recovered from TTP-HUS, deaths still occurred and many patients suffered long-term complications. The role of the treatments given in addition to plasma is uncertain.
Hayward
C P
CP
Department of Hematology, Toronto (Ontario) Hospital.
Sutton
D M
DM
Carter
W H
WH
Campbell
E D
ED
Scott
J G
JG
Francombe
W H
WH
Shumak
K H
KH
Baker
M A
MA
D000293:Adolescent; D000328:Adult; D000368:Aged; D017707:Erythrocyte Transfusion; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000209:etiology / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000628:therapy*; D012008:Recurrence; D017582:Renal Replacement Therapy; D013156:Splenectomy; D016896:Treatment Outcome