Causes and risk factors of death in patients with thrombotic microangiopathies.
OBJECTIVE
Although plasma therapy of thrombotic micro-angiopathies (TMAs) has dramatically improved survival, the outcome remains fatal in up to 15 % of patients. We investigated the causes and risk factors of death in patients with TMA.


METHODS
Retrospective matched case-control national-registry study of 57 patients who died within 180 days of TMA diagnosis and 48 survivors matched on age, gender, and baseline platelet count and creatinine level. The study period was 1995-2007. Factors associated with mortality were identified using a conditional logistic regression model.


RESULTS
Median time from TMA symptom onset to death was 7 (5-14) days. The leading causes of death were nosocomial infections, myocardial infarction, stroke, and pulmonary embolism. Cases and controls did not differ significantly regarding haemolysis parameters, ADAMTS13 activity, or neurological or gastrointestinal involvement. TMA was more frequently related to HIV or cancer in patients who died. Compared to survivors, non-survivors more often had cardiac involvement at diagnosis (38 vs. 6 %, p = 0.03) and less often received plasma exchange therapy (60 vs. 92 %, p = 0.004). Only two factors were independently associated with mortality by multivariate analysis: cardiac involvement at diagnosis (odds ratio, 5.96; 95 % confidence interval, 1.06-33.4) and plasma exchange therapy (odds ratio, 0.25; 95 % confidence interval, 0.06-0.99).


CONCLUSIONS
Our data emphasise the adverse prognostic significance of cardiac abnormalities and support routine plasma exchange in patients with TMA. Given the high risk of cardiac and neurological complications, adequate monitoring should be proposed to these patients in appropriate hospital settings.
AP-HP, Hôpital Saint-Louis Medical ICU, 1 Avenue Claude Vellefaux, 75010, Paris, France.
Vincent
V
Peigne
Pierre
P
Perez
Matthieu
M
Resche Rigon
Eric
E
Mariotte
Emmanuel
E
Canet
Jean-Paul
JP
Mira
Paul
P
Coppo
Agnès
A
Veyradier
Elie
E
Azoulay
D000328:Adult; D016022:Case-Control Studies; D002423:Cause of Death; D015897:Comorbidity; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010951:Plasma Exchange; D012189:Retrospective Studies; D012307:Risk Factors; D057049:Thrombotic Microangiopathies / Q000401:mortality* / Q000628:therapy